Raras
Buscar doenças, sintomas, genes...
Microangiopatia trombótica
ORPHA:93573CID-10 · M31.1DOENÇA RARA

São condições que se manifestam como um tipo de anemia (sangue fraco) onde os glóbulos vermelhos são destruídos em vasos sanguíneos muito pequenos; poucas plaquetas no sangue (células que ajudam a coagular); e diversos sinais de que os órgãos não estão funcionando bem. Isso ocorre porque as plaquetas se agrupam e bloqueiam a circulação nos vasos sanguíneos menores.

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Introdução

O que você precisa saber de cara

📋

São condições que se manifestam como um tipo de anemia (sangue fraco) onde os glóbulos vermelhos são destruídos em vasos sanguíneos muito pequenos; poucas plaquetas no sangue (células que ajudam a coagular); e diversos sinais de que os órgãos não estão funcionando bem. Isso ocorre porque as plaquetas se agrupam e bloqueiam a circulação nos vasos sanguíneos menores.

Publicações científicas
5.880 artigos
Último publicado: 2026 Mar
Medicamentos
13 registrados
ROMIPLOSTIM, DEXAMETHASONE SODIUM PHOSPHATE, DEXAMETHASONE

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13 medicamentos registrados
Ver detalhes, fases e interações →
ROMIPLOSTIMDEXAMETHASONE SODIUM PHOSPHATEDEXAMETHASONEELTROMBOPAG OLAMINECORTISONE ACETATEFOSTAMATINIB DISODIUMPREDNISONEECULIZUMABCAPLACIZUMABDECITABINE
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: M31.1
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
13 sintomas
🫘
Rins
8 sintomas
🫃
Digestivo
5 sintomas
❤️
Coração
5 sintomas
🧠
Neurológico
4 sintomas
📏
Crescimento
3 sintomas

+ 42 sintomas em outras categorias

Características mais comuns

Púrpura
Positividade do anticorpo anti-receptor de hormônio estimulador da tireoide
Hemorragia ocular
Epistaxe
Sangramento gengival
Suscetibilidade a hematomas
86sintomas
Sem dados (86)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 86 características clínicas mais associadas, ordenadas por frequência.

PúrpuraPurpura
Positividade do anticorpo anti-receptor de hormônio estimulador da tireoideAnti-thyroid-stimulating hormone receptor antibody positivity
Hemorragia ocularHemorrhage of the eye
EpistaxeEpistaxis
Sangramento gengivalGingival bleeding

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico5.880PubMed
Últimos 10 anos200publicações
Pico2026137 papers
Linha do tempo
2026Hoje · 2026🧪 1985Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

9 genes identificados com associação a esta condição.

ADAMTS13A disintegrin and metalloproteinase with thrombospondin motifs 13Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
O-glycosylation of TSR domain-containing proteins
MECANISMO DE DOENÇA

Thrombotic thrombocytopenic purpura, hereditary

An autosomal recessive hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.

OUTRAS DOENÇAS (1)
congenital thrombotic thrombocytopenic purpura
HGNC:1366UniProt:Q76LX8
FCGR2CLow affinity immunoglobulin gamma Fc region receptor II-cCandidate gene tested inDesconhecido
FUNÇÃO

Receptor for the Fc region of complexed immunoglobulins gamma. Low affinity receptor. Involved in a variety of effector and regulatory functions such as phagocytosis of immune complexes and modulation of antibody production by B-cells

LOCALIZAÇÃO

CytoplasmCell membrane

EXPRESSÃO TECIDUAL(Ubíquo)
Nervo tibial
17.1 TPM
Tecido adiposo
14.4 TPM
Pulmão
13.5 TPM
Adipose Visceral Omentum
11.4 TPM
Baço
10.5 TPM
OUTRAS DOENÇAS (1)
autoimmune thrombocytopenic purpura
HGNC:15626UniProt:P31995
CFIComplement factor ICandidate gene tested inTolerante
FUNÇÃO

Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. Inhibits these pathways by cleaving three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins (PubMed:17320177, PubMed:7360115). Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces (PubMed:12055245, PubMed:21418

LOCALIZAÇÃO

Secreted, extracellular spaceSecreted

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

Hemolytic uremic syndrome, atypical, 3

An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.

OUTRAS DOENÇAS (6)
complement factor I deficiencyDoyne honeycomb retinal dystrophyatypical hemolytic uremic syndrome with complement gene abnormalityHELLP syndrome
HGNC:5394UniProt:P05156
CFBComplement factor BCandidate gene tested inTolerante
FUNÇÃO

Precursor of the catalytic component of the C3 and C5 convertase complexes of the alternative pathway of the complement system, a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277). The alternative complement pathway acts as an amplification loop that enhances other complement pathways (classical, lectin and GZMK) by promoting formation

LOCALIZAÇÃO

SecretedCell surface

VIAS BIOLÓGICAS (1)
Alternative complement activation
MECANISMO DE DOENÇA

Macular degeneration, age-related, 14

A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.

OUTRAS DOENÇAS (4)
complement factor b deficiencyatypical hemolytic uremic syndrome with complement gene abnormalityage related macular degeneration 14atypical hemolytic-uremic syndrome with B factor anomaly
HGNC:1037UniProt:P00751
CFHComplement factor HCandidate gene tested inAltamente restrito
FUNÇÃO

Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces (PubMed:21285368, PubMed:21317894, PubMed:25402769). Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

Basal laminar drusen

Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.

OUTRAS DOENÇAS (10)
complement factor H deficiencybasal laminar drusendense deposit diseaseDoyne honeycomb retinal dystrophy
HGNC:4883UniProt:P08603
DGKEDiacylglycerol kinase epsilonCandidate gene tested inTolerante
FUNÇÃO

Membrane-bound diacylglycerol kinase that converts diacylglycerol/DAG into phosphatidic acid/phosphatidate/PA and regulates the respective levels of these two bioactive lipids (PubMed:15544348, PubMed:19744926, PubMed:21477596, PubMed:22108654, PubMed:23949095). Thereby, acts as a central switch between the signaling pathways activated by these second messengers with different cellular targets and opposite effects in numerous biological processes (PubMed:15544348, PubMed:8626589). Also plays an

LOCALIZAÇÃO

MembraneCytoplasm

VIAS BIOLÓGICAS (1)
Effects of PIP2 hydrolysis
MECANISMO DE DOENÇA

Nephrotic syndrome 7

A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. NPHS7 is an autosomal recessive form characterized by onset of proteinuria usually in the first decade of life. The disorder is progressive, and some patients develop end-stage renal disease within several years. Renal biopsy typically shows membranoproliferative glomerulonephritis.

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
20.3 TPM
Cerebelo
15.9 TPM
Útero
10.9 TPM
Pituitária
10.6 TPM
Cervix Ectocervix
10.4 TPM
OUTRAS DOENÇAS (2)
immunoglobulin-mediated membranoproliferative glomerulonephritisatypical hemolytic-uremic syndrome with DGKE deficiency
HGNC:2852UniProt:P52429
CD46Membrane cofactor proteinCandidate gene tested inTolerante
FUNÇÃO

Acts as a cofactor for complement factor I, a serine protease which protects autologous cells against complement-mediated injury by cleaving C3b and C4b deposited on host tissue. May be involved in the fusion of the spermatozoa with the oocyte during fertilization. Also acts as a costimulatory factor for T-cells which induces the differentiation of CD4+ into T-regulatory 1 cells. T-regulatory 1 cells suppress immune responses by secreting interleukin-10, and therefore are thought to prevent auto

LOCALIZAÇÃO

Cytoplasmic vesicle, secretory vesicle, acrosome inner membrane

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

Hemolytic uremic syndrome, atypical, 2

An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.

OUTRAS DOENÇAS (3)
HELLP syndromeatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with MCP/CD46 anomaly
HGNC:6953UniProt:P15529
C3Complement C3Candidate gene tested inRestrito
FUNÇÃO

Precursor of non-enzymatic components of the classical, alternative, lectin and GZMK complement pathways, which consist in a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system Non-enzymatic component of C5 convertase (PubMed:28264884, PubMed:31507604, PubMed:3653927, PubMed:3897448). Generated following cleavage by C3 convertase, it covalently attaches to the surface of pathogens, where it acts as an opsonin that ma

LOCALIZAÇÃO

SecretedCell surface

VIAS BIOLÓGICAS (2)
Post-translational protein phosphorylationRegulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
MECANISMO DE DOENÇA

Complement component 3 deficiency

A rare defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis.

OUTRAS DOENÇAS (4)
complement component 3 deficiencyatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with C3 anomalyage related macular degeneration 9
HGNC:1318UniProt:P01024
THBDThrombomodulinCandidate gene tested inDesconhecido
FUNÇÃO

Endothelial cell receptor that plays a critical role in regulating several physiological processes including hemostasis, coagulation, fibrinolysis, inflammation, and angiogenesis (PubMed:10761923). Acts as a cofactor for thrombin activation of protein C/PROC on the surface of vascular endothelial cells leading to initiation of the activated protein C anticoagulant pathway (PubMed:29323190, PubMed:33836597, PubMed:9395524). Also accelerates the activation of the plasma carboxypeptidase B2/CPB2, w

LOCALIZAÇÃO

Membrane

VIAS BIOLÓGICAS (2)
Common Pathway of Fibrin Clot FormationCell surface interactions at the vascular wall
MECANISMO DE DOENÇA

Thrombophilia due to thrombomodulin defect

A hemostatic disorder characterized by a tendency to thrombosis.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Sun Exposed Lower leg
112.0 TPM
Pulmão
110.4 TPM
Skin Not Sun Exposed Suprapubic
108.2 TPM
Artéria coronária
91.3 TPM
Aorta
87.3 TPM
OUTRAS DOENÇAS (3)
thrombomodulin-related bleeding disorderatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with thrombomodulin anomaly
HGNC:11784UniProt:P07204

Medicamentos e terapias

ROMIPLOSTIMPhase 4

Mecanismo: Thrombopoietin receptor agonist

DEXAMETHASONE SODIUM PHOSPHATEPhase 4

Mecanismo: Glucocorticoid receptor agonist

DEXAMETHASONEPhase 4

Mecanismo: Glucocorticoid receptor agonist

ELTROMBOPAG OLAMINEPhase 4

Mecanismo: Thrombopoietin receptor agonist

CORTISONE ACETATEPhase 4

Mecanismo: Glucocorticoid receptor agonist

FOSTAMATINIB DISODIUMPhase 4

Mecanismo: Tyrosine-protein kinase SYK inhibitor

PREDNISONEPhase 4

Mecanismo: Glucocorticoid receptor agonist

ECULIZUMABPhase 4

Mecanismo: Complement C5 inhibitor

CAPLACIZUMABPhase 4

Mecanismo: von Willebrand factor inhibitor

DECITABINEPhase 3

Mecanismo: DNA (cytosine-5)-methyltransferase 3A inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

441 variantes patogênicas registradas no ClinVar.

🧬 ADAMTS13: NM_139027.6(ADAMTS13):c.3548-2A>C ()
🧬 ADAMTS13: NM_139027.6(ADAMTS13):c.3G>A (p.Met1Ile) ()
🧬 ADAMTS13: NM_139027.6(ADAMTS13):c.3400+36dup ()
🧬 ADAMTS13: NM_139027.6(ADAMTS13):c.3442dup (p.Asp1148fs) ()
🧬 ADAMTS13: NM_139027.6(ADAMTS13):c.1A>G (p.Met1Val) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 5 variantes classificadas pelo ClinVar.

3
1
1
Patogênica (60.0%)
VUS (20.0%)
Benigna (20.0%)
VARIANTES MAIS SIGNIFICATIVAS
ATRIP: NM_033629.6(TREX1):c.830_833dup (p.Asp278fs) [Likely pathogenic]
CD46: NM_172351.3(CD46):c.287-2A>G [Pathogenic/Likely pathogenic]
CFI: NM_000204.5(CFI):c.1322A>G (p.Lys441Arg) [Conflicting classifications of pathogenicity]
CFH: NM_000186.4(CFH):c.2753G>A (p.Gly918Glu) [Uncertain significance]
CFH: NM_000186.4(CFH):c.3148A>T (p.Asn1050Tyr) [Benign/Likely benign]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado9
3Fase 32
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 10 medicamentos · 2 ensaios
✓ Aprovados — podem ser usados hoje
ROMIPLOSTIMDEXAMETHASONE SODIUM PHOSPHATEDEXAMETHASONEELTROMBOPAG OLAMINECORTISONE ACETATEFOSTAMATINIB DISODIUMPREDNISONEECULIZUMABCAPLACIZUMAB
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Microangiopatia trombótica

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Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

616 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
3.994 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 3.994

#1

[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].

Zhonghua er ke za zhi = Chinese journal of pediatrics2026 Mar 14

Objective: To evaluate the efficacy and safety of eculizumab (ECZ) in pediatric atypical hemolytic uremic syndrome (aHUS). Methods: A single-center retrospective study was conducted on 11 children with aHUS who received ECZ therapy in the Department of Nephrology, Children's Hospital of Fudan University, between December 2022 and February 2025. The clinical data, including genetic testing results, were collected for analyzing hematological and renal remission, prognosis and adverse events. Results: Among the 11 patients, 6 were male and 5 were female, with age at onset of 2.83 (0.55, 5.75) years. Eight patients had newly diagnosed aHUS, and 3 had relapsed disease. The pathogenic genetic variants were identified in 3 patients. Before ECZ treatment, 8 children required dialysis, including 2 with chronic dialysis. The age at first ECZ administration was 5.03 (0.72, 11.39) years, and the follow-up duration was (15±9) months. All 11 patients achieved complete thrombotic microangiopathy remission, with a time to remission of (36±23) d. In the 11 patients, the estimated glomerular filtration rate at the final follow-up increased by 46 (31, 62) ml/(min·1.73 m2) compared with baseline. The time to achieve normalization of serum creatinine or a ≥25% reduction was 10.0 (5.5, 31.5) d. Plasma exchange or plasma infusion was discontinued in all patients. The 8 patients who required dialysis at baseline successfully discontinued dialysis, and no new dialysis cases occurred. Six patients with negative genetic testing discontinued ECZ without relapse during follow-up. ECZ was well tolerated, with no meningococcal infections reported. Two patients with elevated liver enzymes recovered fully. Conclusions: ECZ demonstrates favorable efficacy and tolerability in pediatric aHUS. The application of ECZ may contribute to improve the prognosis of aHUS. 目的: 探讨依库珠单抗(ECZ)治疗儿童非典型溶血尿毒综合征(aHUS)的疗效和安全性。 方法: 病例系列研究,收集2022年12月至2025年2月在复旦大学附属儿科医院肾内科应用ECZ治疗的11例aHUS患儿临床资料包括基因诊断,分析其血液学、肾脏缓解情况以及预后和不良反应。 结果: 11例患儿中,男6例、女5例,起病年龄2.83(0.55,5.75)岁;8例为新发病例,3例为复发病例;3例检出aHUS相关基因变异。ECZ治疗前有8例患儿需行透析治疗,其中2例为长期透析患儿。首剂ECZ治疗年龄为5.03(0.72,11.39)岁,随访时间(15±9)个月。ECZ治疗后,11例患儿均达到血栓性微血管病完全缓解,达到时间(36±23)d。11例患儿末次随访估算肾小球滤过率较基线上升了46(31,62)ml/(min·1.73 m2);达到血肌酐正常或下降≥25%的时间为10.0(5.5,31.5)d。所有患儿均停止血浆置换或血浆输注;基线时需要透析的8例患儿均成功停止透析,无新增透析患儿。6例基因检测阴性患儿已停用ECZ,无复发病例。ECZ耐受性良好,没有发生脑膜炎球菌感染;2例肝酶升高患儿均恢复正常。 结论: ECZ对儿童aHUS具有良好的疗效和耐受性。应用ECZ有助于改善aHUS预后。.

#2

Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.

JAMA network open2026 Mar 02

Shiga toxin-producing Escherichia coli (STEC) infections have significant morbidity, with 15% to 20% of children developing hemolytic uremic syndrome (HUS), more than half of whom require kidney replacement therapy (KRT). To describe health care resource utilization, clinical outcomes, and outcomes associated with daily laboratory monitoring for thrombotic microangiopathy (TMA) in a large point-source STEC outbreak. This retrospective cohort study was conducted among a population-based cohort of individuals exposed to a contaminated food item served in 11 childcare centers in Calgary, Alberta, Canada, on August 29, 2023. Children and adults with confirmed STEC infection linked to the outbreak were included. Data were analyzed from June 9, 2025, to January 5, 2026. E coli O157:H7 possessing genes encoding Shiga toxins 1 and 2. Health care utilization, symptoms and signs of infection, occurrence of HUS and KRT, and results of TMA screening. There were 359 outbreak-linked confirmed STEC infections, including 285 children (median [IQR] age, 3.3 [2.3-4.2] years; 141 [62.4%] male) and 71 adults (median [IQR] age, 38.8 [31.9-46.4] years; 58 [81.7%] female) and 3 out-of-province cases; 326 primary and 33 secondary infections were identified. Of 359 infected individuals overall, 66 (18.4%) were asymptomatic. HUS occurred in 21 children (7.4%), all of whom had diarrhea (21 of 206 children with diarrhea [10.2%]). No adults developed HUS. This outbreak was linked to 508 and 395 visits by children with STEC to an emergency department and a dedicated STEC clinic, respectively; 591 visits occurred during a 7-day period, starting 6 days after exposure. Forty children were hospitalized, 9 children required KRT, and 3 children required intensive care. The most common symptoms in children and adults were diarrhea (206 children [91.2%]; 16 of 20 adults with data [80.0%]), abdominal pain (141 children [62.4%]; 11 of 20 adults with data [55.0%]), and bloody diarrhea (127 children [56.2%]; 7 of 20 adults with data [35.0%]). TMA testing had 100% (95% CI, 83.9%-100%) sensitivity, 95.7% (95% CI, 92.0%-98.0%) specificity, and a diagnostic accuracy of 96.1% (95% CI, 92.7%-98.2%) for development of HUS. This cohort study reporting on a point-source STEC outbreak identified significant use of health care resources. Only 7% of children infected with STEC developed HUS, which is lower than reported in most prior outbreaks and sporadic case series. These findings highlight the importance of a coordinated public health response integrated with clinical care and suggest that proactive screening for TMA has the potential to mitigate adverse outcomes.

#3

Role of extracorporeal therapy in poisoning - A case based narrative.

International journal of critical illness and injury science2026

Extracorporeal treatment (ECTR) have been employed for poison elimination dating back to as early as the 1960s, but during the current day and time, the indications for ECTR are mostly arbitrary and are considered when other treatments of poison elimination or reduction below toxic levels are not available. It is invaluable, especially when the toxins or poison are amendable to removal by ECTR, especially in a setting of clinical deterioration, the unavailability of specific antidotes, or when other treatment modalities are cost prohibitive in a tertiary setting with ECTR facilities. To reiterate the efficacy of extracorporeal therapy techniques in poisoning focusing on the indications, available modalities, and outcomes. Three clinical cases of acute poisoning with phenobarbitone, copper sulfate, and snake envenomation, effectively managed by ECTR, in detail were reviewed. A literature review on ECTR in poisoning was also conducted to summarize the principles. All three patients were managed with appropriate ECTR modalities. Therapeutic plasma exchange was used to treat snakebite-induced thrombotic microangiopathy, hemodialysis was used to treat phenobarbital toxicity, and plasma exchange was used effectively for copper sulfate poisoning. ECTR was initiated early, which resulted in clinical improvement and recovery in all cases. HD remains a pivotal treatment option for the management of severe poisonings. Nevertheless, given the rare possibility of an encounter with cases necessitating treatment with extracorporeal therapies and the lack of recommendations and guidelines to standardize the practice, the evidence supporting the application of ECTR still remains limited and needs to be studied further for high-quality evidence.

#4

Presentation of HIV-Associated Thrombotic Microangiopathy and Response to Therapeutic Plasma Exchange: A 10-year Retrospective Single-Centre Cohort Study.

Health science reports2026 Mar

HIV is a significant aetiological factor in thrombotic microangiopathy (TMA) in regions of high seroprevalence, but description of HIV-associated TMA (HIV-TMA) remains limited to small case series. We sought to describe the presentation, complications of TPE, and mortality and renal outcomes of HIV-TMA. We retrospectively reviewed 98 cases of HIV-TMA treated with therapeutic plasma exchange (TPE) between 1/1/2010 and 31/12/2020. The effect of HIV infection and clinical presentation on mortality, TPE complications, and renal outcomes were analysed using regression analysis. TMA is associated with advanced HIV infection (median CD4 count 151 × 106/mm3 cells and median viral load 117500 copies/mL), usually occurs in the absence of antiretroviral therapy (ART), and shows a predilection for young Black women, reflecting TMA risk factors and local demographics of the HIV pandemic. Neurological deficit is the most common presenting feature (54.1%). HIV-TMA mortality is high despite TPE (49% of cases); better renal function reduces risk of TMA-attributable death (HR 0.97, 95% CI 0.96-0.99, p < 0.001); use of FFP as infusant is associated with increased risk of mortality (HR 3.96, 95% CI 1.60-9.84, p = 0.003). Sepsis frequently complicates TPE (16.3% of courses) and contributes to excess mortality (20.7% of deaths); risk of infection increases with duration of TPE (OR 1.21, 95% CI 1.07-1.37, p = 0.002), implicating augmented immunosuppression in mortality. HIV infection parameters do not significantly affect risk of mortality or sepsis. Residual renal dysfunction is relatively rare in survivors at follow-up. Mortality remains high in HIV-TMA treated with TPE, and sepsis-related complications are of concern. Randomized prospective studies are needed to evaluate the use of TPE versus plasma infusion (PI) and infusant choice in HIV-TMA. Longer duration follow-up studies are needed to evaluate residual renal dysfunction in survivors of HIV-TMA.

#5

Reinventing the wheel in ischemic stroke: Novel aspects in ADAMTS13 and von Willebrand axis.

Blood reviews2026 Feb 25

A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), is an enzyme that cleaves ultra-large von Willebrand factor (VWF) multimers. ADAMTS13 deficiency leads to thrombotic thrombocytopenic purpura (TTP), a thrombotic microangiopathy characterized by micro-thrombosis in small vessels and target-organ damage. Given that in long-term TTP survivors, reduced ADAMTS13 activity has been associated with the development of major cardiovascular events, the emerging evidence regarding the dysregulation of the ADAMTS13/ VWF axis in the pathogenesis of ischemic stroke (IS), and the unmet need for novel predictive models for IS outcomes and novel targeted therapeutics, we aim to review the current literature to provide an overview of the ongoing research on this field. Our objective is to summarize the evidence from experimental and clinical studies to underline the pathogenetic and therapeutic potential of VWF-ADAMTS13 in IS, while also providing perspectives for future research in this domain.

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📚 EuropePMC2.484 artigos no totalmostrando 197

2026

Safety and Efficacy of Therapeutic Plasma Exchange in Pediatric Non-Neurological Diseases.

International journal of nephrology and renovascular disease
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Early recognition of atypical hemolytic uremic syndrome to prevent irreversible kidney injury: cardiac failure and refractory hypertension as critical clues in young patients.

CEN case reports
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Recurrent pancreatitis and atypical hemolytic uremic syndrome (aHUS): an unusual presentation in childhood.

Pediatric nephrology (Berlin, Germany)
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Expression of Human Thrombomodulin Prevents Early Thrombocytopenia and Thrombotic Microangiopathy in Pig-to-Nonhuman Primate Orthotopic Liver Xenotransplantation.

Xenotransplantation
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Erythrodermic psoriasis complicated by immune complex-mediated crescentic glomerulonephritis and atypical hemolytic uremic syndrome: a case report.

BMC nephrology
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Beyond HELLP: An Unusual Cause of Severe Thrombocytopenia in Pregnancy.

Cureus
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Hemoglobinuria-associated acute kidney injury in hemolytic uremic syndrome without renal thrombotic microangiopathy.

BMC nephrology
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Pulmonary Tumor Thrombotic Microangiopathy in a Patient With Male Breast Cancer: A Report of a Rare Case.

Cureus
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Late-Onset Normotensive Thrombotic Microangiopathy and Pyoderma Gangrenosum Following Nine Years of Sunitinib Therapy: A Case Report.

Kidney medicine
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Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.

Journal of thrombosis and haemostasis : JTH
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Efficacy analysis of iptacopan in a patient with thrombotic microangiopathy after allogeneic hematopoietic stem cell transplantation: a case report.

Frontiers in immunology
2026

Thrombotic microangiopathy with features of thrombotic thrombocytopenic purpura in a patient with Vibrio parahaemolyticus bacteremia: a rare case report.

Internal and emergency medicine
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[Once upon a time in nephrology - Episode 3: A brief history of preeclampsia].

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Severe systemic lupus erythematosus with thrombotic microangiopathy: rescue therapy with pegcetacoplan.

Pediatric nephrology (Berlin, Germany)
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Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.

Frontiers in immunology
2026

Recurrent Atypical Hemolytic-Uremic Syndrome (aHUS) Associated With CD46 Genetic Mutation: A Report of a Rare Case.

Cureus
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[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].

Zhonghua er ke za zhi = Chinese journal of pediatrics
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Clinical kidney journal
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Dynamic Monitoring of ADAMTS-13 Activity for Differential Diagnosis Across the Spectrum of Sepsis-Associated Thrombotic Microangiopathies.

Mediterranean journal of hematology and infectious diseases
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A Prospective 1-Year Study of Renal Recovery in Pigment Nephropathy: Insights Beyond the Acute Phase.

The Journal of the Association of Physicians of India
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Surgical case reports
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The C3 p.Ile1157Thr mutation associated with atypical hemolytic uremic syndrome, particularly in Japan, does not lead to disease development in several mouse models.

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PeerJ
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Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.

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Long-Term Risk of Major Coronary Artery Disease Events After Thrombotic Microangiopathy Treated With Plasma Exchange.

American journal of hematology
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International journal of critical illness and injury science
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Presentation of HIV-Associated Thrombotic Microangiopathy and Response to Therapeutic Plasma Exchange: A 10-year Retrospective Single-Centre Cohort Study.

Health science reports
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Immunoglobulin A Nephropathy With Associated Thrombotic Microangiopathy: Biopsy and Clinical Case Series.

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Pediatric nephrology (Berlin, Germany)
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Expert review of hematology
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Case reports in rheumatology
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Can low serum complement be used as an indicator of thrombotic microangiopathy in kidney transplant candidates with unknown diagnosis?

Journal of nephrology
2026

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Therapeutic advances in hematology
2026

A young woman with membranoproliferative glomerulonephritis and persistent hypocomplementemia: a case report.

BMC nephrology
2026

Reinventing the wheel in ischemic stroke: Novel aspects in ADAMTS13 and von Willebrand axis.

Blood reviews
2026

Detecting Endothelial Compromise Objectively through Dynamic EASIX scoring (DECODE) - predictive utility of EASIX and modified EASIX scores for endothelial complications post hematopoietic cell transplant.

Frontiers in oncology
2026

Pregnancy-Associated Thrombotic Thrombocytopenic Purpura: Diagnostic Pitfalls, Therapeutic Strategies, and Emerging Paradigms.

Biomedicines
2026

Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.

Journal of the American Academy of Dermatology
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Late Onset of Congenital TTP: Case Presentation and Review of the Literature.

Clinical medicine insights. Case reports
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Validation of D-Dimer as a Prognostic Biomarker in Transplant-Associated Thrombotic Microangiopathy in Children.

Pediatric blood &amp; cancer
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Pathologic diagnosis incorporating molecular monitoring highlights new pathophysiological mechanisms in kidney xenotransplantation.

Kidney international
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Cureus
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Annals of hematology
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Frontiers in oncology
2026

Case Report: CAR-T cell therapy bridging to allogeneic hematopoietic stem cell transplantation triggers Purtscher-like retinopathy: clinical features and complement-mediated microvascular injury mechanisms.

Frontiers in immunology
2026

Successful living kidney transplantation in a T-cell flow cytometry crossmatch-positive patient with atypical hemolytic uremic syndrome treated with an anti-C5 antibody: a case report.

CEN case reports
2026

Genetics of thrombotic thrombocytopenic purpura: systematic review in immune and congenital thrombotic thrombocytopenic purpura.

Journal of thrombosis and haemostasis : JTH
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Identifying novel biomarkers for systemic lupus erythematosus associated with macrophage activation syndrome.

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
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Journal of neurology
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BMC pulmonary medicine
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Multisystem involvement with ischemic complications in a child with STEC-HUS: a case of gangrene.

Pediatric nephrology (Berlin, Germany)
2026

Early eculizumab treatment improves renal outcomes in pediatric lupus nephritis with thrombotic microangiopathy.

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
2026

C3 mutation-associated atypical hemolytic uremic syndrome with severe renal dysfunction and hypertensive emergency successfully treated with ravulizumab and sacubitril/valsartan: a case report.

BMC nephrology
2025

Linking preclinical models to clinical realities: VEGF/VEGFR inhibitors and thrombotic microangiopathy in cancer therapy.

iMetaOmics
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Impact of Day of Hospital Admission and Hospital Characteristics on Mortality in Thrombotic Thrombocytopenic Purpura.

Journal of clinical apheresis
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Syndrome of Inappropriate Antidiuretic Hormone Secretion and Thrombotic Microangiopathy as Paraneoplastic Syndromes Complicating BRCA2-Mutated Metastatic Prostate Cancer.

Cureus
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Acute Kidney Injury Following Vaccine-Induced Thrombotic Microangiopathy.

European journal of case reports in internal medicine
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Actinomycotic Cholecystitis and Pancreatitis: Report of an Unusual Case.

The American journal of case reports
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Advanced Pediatric HIV Presenting as Thrombotic Microangiopathy.

Indian journal of pediatrics
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Hypertensive Havoc: When Malignant Hypertension Mimics Thrombotic Thrombocytopenic Purpura.

Cureus
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Case Report: Glomerular microangiopathy and reversible renal toxicity during prolonged bevacizumab therapy in ovarian cancer.

Frontiers in oncology
2026

Severe venom-induced consumption coagulopathy, snakebite-associated thrombotic microangiopathy, and local necrosis following Western bush viper (Atheris chlorechis) envenoming in France.

Toxicon : official journal of the International Society on Toxinology
2026

RTX-family toxin EhxA drives morphological remodeling and thrombogenesis in RBCs during enterohemorrhagic Escherichia coli infection.

Science advances
2026

Long-chain polyphosphates induce glomerular microthrombi and exacerbate LPS-induced acute kidney injury in mouse.

Disease models &amp; mechanisms
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A case of membranous proliferative glomerulonephritis-type IgA nephropathy modified by nintedanib.

CEN case reports
2026

Fibronectin glomerulopathy caused by genetic FN1 mutation: A case report and literature review.

Clinical nephrology
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Optimizing the management of congenital thrombotic thrombocytopenic purpura.

Research and practice in thrombosis and haemostasis
2026

Delayed-Onset Hemolysis in a Case of Hemolytic Uremic Syndrome: A Diagnostic Challenge.

Case reports in hematology
2026

Efficacy of Plasma Exchange in Secondary Thrombotic Microangiopathy: A Case Report and Literature Review.

Cureus
2026

Chronic health manifestations following hump-nosed pit viper (Hypnale spp.) bites: an authenticated case series.

Transactions of the Royal Society of Tropical Medicine and Hygiene
2026

Acute kidney injury requiring temporary hemodialysis due to HELLP syndrome: a case report.

CEN case reports
2026

Real-World Effectiveness of Ravulizumab Among C5 Inhibitor-Naive Patients With Atypical Hemolytic Uremic Syndrome: A Physician Panel-Based Chart Review (aHUS IMPACT Study).

Kidney medicine
2026

Acute myocardial infarction following allogeneic hematopoietic stem cell transplantation: A national cohort study.

Cancer letters
2025

Eculizumab in treatment for complement-mediated thrombotic microangiopathy associated with acute pancreatitis.

EXCLI journal
2026

Caveolin-1 as a Marker of Endothelial Damage in Primary Antiphospholipid Syndrome Nephropathy.

Kidney medicine
2026

Macrophage Activation Syndrome-Associated Proteins and Enhanced Interferon-γ Responsiveness in the Plasma Proteome of Patients With Multisystem Inflammatory Syndrome in Children in a Pretreatment Replication Single-Center Cohort.

ACR open rheumatology
2026

Clinical characteristics and outcome of malaria-associated acute kidney injury in adult patients: a systematic review and meta analysis.

Malaria journal
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Bone marrow proteomic profiling reveals TMEM109 as a biomarker for relapse in thrombotic thrombocytopenic purpura.

Journal of thrombosis and haemostasis : JTH
2026

Thrombotic microangiopathy in a patient with anti-signal recognition particle antibody-positive immune-mediated necrotising myopathy: a case report.

Modern rheumatology case reports
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Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.

Frontiers in immunology
2026

[Management of thrombotic microangiopathy].

Revue medicale suisse
2025

Cutaneous and Systemic Complications in Primary CD8+ Aggressive Epidermotropic Cytotoxic T-cell Lymphoma.

Cureus
2025

Safety of Post-transplant Cyclophosphamide as a Single Agent for Graft-Versus-Host Disease Prophylaxis After Human Leukocyte Antigen (HLA)-Matched Transplantation With the Japanese Population: A Single-Center Phase Ⅰ/Ⅱ Study.

Cureus
2026

Successful term pregnancy after renal transplant in end-stage renal disease with complement factor H-related mutation: A case report.

World journal of transplantation
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From dog bite to dialysis: complement-mediated haemolytic uraemic syndrome .

BMJ case reports
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Catastrophic Antiphospholipid Syndrome.

Seminars in thrombosis and hemostasis
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Primary antiphospholipid syndrome presenting with unilateral renal infarction and C4d-positive cortical necrosis: a case report with a pooled analysis of 24 cases.

Frontiers in immunology
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Long-term renal outcomes of patients with biopsy-proven malignant hypertension-associated renal thrombotic microangiopathy who are dialysis-independent at baseline.

Renal failure
2026

Relationship between kidney length and renal risk in malignant hypertension patients with renal thrombotic microangiopathy.

Clinical and experimental hypertension (New York, N.Y. : 1993)
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Salvaging sepsis-associated atypical hemolytic uremic syndrome with terminal complement blockade: A case report.

Medicine
2025

[Microangiopathies thrombotiques].

La Revue du praticien
2026

Monitoring ADAMTS-13 conformation in immune-mediated thrombotic thrombocytopenic purpura: toward personalized management.

Research and practice in thrombosis and haemostasis
2025

Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus after anifrolumab: a possible association.

Archive of clinical cases
2026

Thrombotic microangiopathy or rejection in first pig-to-human patient liver xenotransplantation.

Journal of hepatology
2026

Health-related quality of life and mental health in autoimmune thrombotic thrombocytopenic purpura patients in the caplacizumab era.

Research and practice in thrombosis and haemostasis
2026

Survival after cardiopulmonary arrest due to Capnocytophaga canimorsus sepsis: A case successfully managed using plasma exchange.

IJID regions
2026

Use of narsoplimab for eculizumab-refractory adult transplant-associated thrombotic microangiopathy (TA-TMA).

Annals of hematology
2026

Clinicopathological Spectrum and Treatment Outcomes of Cryofibrinogen-Associated Nephropathies.

Kidney international reports
2026

Bicistronic-CD19/22 Chimeric Antigen Receptor T-Cell Therapy for Childhood Refractory Lupus Nephritis.

Kidney international reports
2026

Postpartum Readmission for Pregnancy-Associated Thrombotic Microangiopathy Is Associated With Poor Maternal Outcomes.

Journal of clinical apheresis
2025

Thrombotic Microangiopathy Secondary to Capnocytophaga Sepsis: A Case Report.

Cureus
2026

Retrospective cohort evaluation of renal involvement in non-HIV castleman disease patients from a single academic center in Beijing, China.

Annals of hematology
2026

From uterine atony to aHUS: A case of postpartum thrombotic microangiopathy.

Revista espanola de anestesiologia y reanimacion
2026

Acyclovir-Resistant Herpes Simplex Virus in Pediatric Patients Undergoing Allogeneic Hematopoietic Stem Cell Transplant: A Case Series.

Transplant infectious disease : an official journal of the Transplantation Society
2026

Biopsy-proven tyrosine kinase inhibitor-associated renal injury: a case series.

Clinical kidney journal
2026

Thrombotic Microangiopathy and Glomerulonephritis Complicating Staphylococcus aureus-Induced Impetigo in a Girl.

The Pediatric infectious disease journal
2026

Incidence and Impact of Endothelial Dysfunction Events and Veno-Occlusive Disease in Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide: A Retrospective Analysis From the Argentinian Group of Marrow Transplant and Cellular Therapy (GATMO-TC).

Transplantation and cellular therapy
2026

Incidence, timing, and clinical significance of adverse immune events after gene replacement therapy: A systematic review and meta-analysis.

Molecular therapy : the journal of the American Society of Gene Therapy
2026

Diagnostic and therapeutic challenges into snakebite-induced thrombotic microangiopathy: a case report and review of the literature.

Journal of medical case reports
2025

Risk Factors Associated with the Development of Thrombotic Microangiopathy in Patients with Dermatomyositis.

International journal of molecular sciences
2026

Validation of risk stratification and novel prognostic biomarkers in asian adult HSCT recipients with TA-TMA: A multicenter real-world study.

Bone marrow transplantation
2026

Diagnostic Challenges in Atypical Hemolytic Uremic Syndrome: A Case of Artificial Mitral Valve Dysfunction as a Possible Trigger for Severe Thrombotic Microangiopathy.

The American journal of case reports
2026

Ethnicity affects relapse-free survival in immune-mediated thrombotic thrombocytopenic purpura.

Haematologica
2026

Pulmonary tumour thrombotic microangiopathy or chronic thromboembolic pulmonary hypertension?-diagnosed by pulmonary wedge aspiration cytology using a Swan-Ganz catheter: a case report.

European heart journal. Case reports
2026

Crovalimab Rescue Therapy in a Case With Genetic Complement Mediated Thrombotic Microangiopathy.

Kidney medicine
2026

Petting Zoo Perils: Escherichia coli O157:h7 Associated Hemolytic Uremic Syndrome Secondary to Animal Exposure.

Hemodialysis international. International Symposium on Home Hemodialysis
2026

Diagnosis and treatment of complement-mediated thrombotic microangiopathies: consensus of the Genetic Diseases Committee of the Chilean Society of Nephrology.

BMC nephrology
2026

Functional Xenogeneic Liver Support in a Living Human: Promise, Lessons, and Next Steps.

Xenotransplantation
2026

[Targeting complement in atypical hemolytic and uremic syndrome: development and current status of eculizumab and ravulizumab].

Nephrologie &amp; therapeutique
2026

A milestone in liver xenotransplantation: The first 10-gene-edited pig-to-living-human auxiliary transplantation and the road ahead.

Cell transplantation
2025

Acute kidney injury associated with acute fatty liver of pregnancy: An update on a rare clinical entity.

World journal of nephrology
2026

Mechanistic insights from transcript analysis of long-term pig to non-human primate kidney xenografts.

Kidney international
2025

Atypical Hemolytic Uremic Syndrome After Post-Abortion Infection: Case Report and Literature Review.

International journal of women's health
2026

The Era of Gene Therapy, Newborn Screening, and Improved Management in the Leukodystrophies: A Shifting Framework With Altered Expectations.

Pediatric neurology
2025

Renal Autotransplantation After Yang-Monti Neoureter Procedure: Surgical Case Report and Brief Literature Review.

Case reports in urology
2025

Postoperative Bilateral Renal Cortical Necrosis Secondary to Antiphospholipid Syndrome: A Case Report and Literature Review.

Canadian journal of kidney health and disease
2025

Liver Involvement in Children with Hemolytic Uremic Syndrome: Clinical Significance and Prognostic Value.

Children (Basel, Switzerland)
2025

Genotype-Phenotype Variability in Congenital Thrombotic Thrombocytopenic Purpura (TTP): Two Novel ADAMTS13 Variants.

Cureus
2026

Nephrotic syndrome genomic discovery in the Mass General Brigham Biobank identifies monoallelic MEFV variants as a risk factor for focal segmental glomerulosclerosis.

Kidney international
2025

[Pulmonary Tumor Thrombotic Microangiopathy - Case Report].

Harefuah
2025

Clinical differential analysis of severe complications thrombotic microangiopathy and acute graft-versus-host disease following allogeneic hematopoietic stem cell transplantation.

Frontiers in oncology
2025

Lipid dysregulation after hematopoietic stem cell transplant.

Haematologica
2026

Pregnancy-related acute kidney injury in south India: Clinical profile and maternal outcomes.

Tropical doctor
2025

Outcomes of allogeneic hematopoietic stem cell transplantation for acquired pure red cell aplasia.

International journal of hematology
2025

Post-miscarriage Complement-Mediated Thrombotic Microangiopathy in a 27-Year-Old Woman: A Case Highlighting Diagnostic and Therapeutic Gaps in Brazil.

Cureus
2026

Temporal biomarker dynamics and clinical outcomes in transplant-associated thrombotic microangiopathy (TA-TMA).

Transplantation and cellular therapy
2026

Tick saliva molecules as potential immunomodulatory therapeutics.

Parasitology international
2025

The Patient-Reported Outcomes Measurement Information System perspective of adults with long-standing atypical hemolytic uremic syndrome.

Research and practice in thrombosis and haemostasis
2025

Gene therapy in Duchenne muscular dystrophy.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
2026

Thrombotic thrombocytopenic purpura following allogeneic hematopoietic stem cell transplantation: a rare but fatal complication.

Journal of thrombosis and haemostasis : JTH
2026

In patients with suspected thrombotic thrombocytopenic purpura, what is the optimal time to therapeutic plasma exchange?

Hematology, transfusion and cell therapy
2026

Post‑Marketing Surveillance of the Safety and Effectiveness of Emicizumab in Japanese Patients With Congenital Haemophilia A With Inhibitors.

Haemophilia : the official journal of the World Federation of Hemophilia
2025

Thrombotic thrombocytopaenic purpura.

European heart journal. Case reports
2025

Hemolytic Uremic Syndrome Complicated by Severe Neuropsychiatric Symptoms: A Case Report and Review of the Literature.

Cureus
2026

Mammalian target of rapamycin inhibitors as the treatment for steroid-refractory acute graft-vs.-host disease after allogeneic hematopoietic stem cell transplantation: A systematic review and individual patient data meta-analysis.

Experimental and therapeutic medicine
2025

[The night has many faces: When schistocytes step out of line].

Praxis
2025

Ravulizumab in treatment-naïve patients with atypical hemolytic uremic syndrome: a real-world case series.

Drug target insights
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Pathologic findings in preclinical and early clinical kidney xenotransplantation.

Current opinion in organ transplantation
2025

Enterohemorrhagic Escherichia coli O157 outer membrane vesicles administered by oral gavage cause renal tubular injury and acute kidney failure in mice.

Frontiers in cellular and infection microbiology
2025

Late Onset Thrombotic Microangiopathy in Kidney Transplants; Poor Outcome Despite Eculizumab Treatment.

Transplant international : official journal of the European Society for Organ Transplantation
2026

Thrombomodulin protects against acute vascular and multiorgan injury in sickle cell disease.

JCI insight
2026

Feasibility and Diagnostic Utility of Mucosal T-Cell Flow Cytometry for Intestinal Graft-Versus-Host Disease.

Gastro hep advances
2025

Renal thrombotic microangiopathy impairs renal function recovery in lupus nephritis.

Clinical kidney journal
2026

Suboptimal management of cancer-associated thrombotic microangiopathies in newly diagnosed and known cancers: A 15-year provincial retrospective cohort study.

Thrombosis research
2026

Thrombotic Microangiopathy During Pregnancy: Role of Soluble Fms-like Tyrosine Kinase-1-Placental Growth Factor Ratios.

American journal of kidney diseases : the official journal of the National Kidney Foundation
2025

Bridging via extracorporeal cardiopulmonary resuscitation for hemodynamic collapse in a patient with metastatic lung cancer: a case report.

JA clinical reports
2025

Tailored treatment of acute immune-mediated thrombotic thrombocytopenic purpura.

Hematology. American Society of Hematology. Education Program
2025

Long-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.

Hematology. American Society of Hematology. Education Program
2025

Modern management of congenital thrombotic thrombocytopenic purpura (cTTP).

Hematology. American Society of Hematology. Education Program
2025

Update in the diagnosis of complement-mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome.

Hematology. American Society of Hematology. Education Program
2025

The conundrum of thrombotic microangiopathy in pregnancy.

BMJ case reports
2025

Case Report: Gemcitabine-induced membranoproliferative glomerulonephritis with immune complexes in a patient with metastatic pancreatic cancer.

Frontiers in medicine
2025

EASIX, a new tool to predict response and refractoriness in immune-mediated thrombotic thrombocytopenic purpura.

Frontiers in immunology
2025

Terminal complement inhibition in atypical haemolytic uremic syndrome: a single-centre experience.

Frontiers in pharmacology
2026

Immune-mediated thrombotic thrombocytopenic purpura as a model of systemic microvascular dysfunction: moving from an acute to a chronic disorder.

Journal of thrombosis and haemostasis : JTH
2025

Outcomes of Terminal Complement Blockade in Adults with High-Risk Transplant-Associated Thrombotic Microangiopathy: A Comparative Analysis.

Transplantation and cellular therapy
2026

Navigating complexities: differential diagnosis of thrombotic microangiopathies in pregnancy - a case report.

Acta clinica Belgica
2025

Pulmonary Tumor Thrombotic Microangiopathy in Platinum-Resistant Ovarian Cancer - A Case Report.

Acta Cardiologica Sinica
2025

Predicting Outcomes and Optimizing Therapy in Thrombotic Thrombocytopenic Purpura: Insights on Caplacizumab Use from a Romanian Hematology Center.

Journal of clinical medicine
2025

Characterization of a Long-Acting Anti-Human MASP-2 Antibody for the Treatment of Complement-Related Diseases.

Journal of inflammation research
2025

Thrombotic Thrombocytopenic Purpura During Anti-Tuberculosis Therapy: A Case Report and Literature Review.

Infection and drug resistance
2025

Anticancer drug-induced nephrotoxicity: biopsy-proven patterns and outcomes across chemotherapy, targeted therapy, and immune checkpoint inhibitors.

Renal failure
2025

Navigating Pediatric Atypical Hemolytic Uremic Syndrome: A Two-Year Case Series From Eastern India.

Cureus
2026

Eculizumab as an adjunctive rescue treatment for life-threatening childhood onset of systemic lupus erythematosus.

Lupus
2026

Bicytopenia with haemolysis: Think severe vitamin B12 deficiency-associated thrombotic microangiopathy.

Tropical doctor
2025

Gemcitabine-Induced Thrombotic Microangiopathy in a Patient With Metastatic Pancreatic Cancer: A Case Report.

Cureus
2025

Shiga Toxin-Producing Escherichia coli-Associated Hemolytic Uremic Syndrome in Adult Kidney Transplant Recipients.

Kidney international reports
2025

Antiphospholipid Syndrome and the Kidney.

Kidney international reports
2025

Ocular manifestations of primary thrombotic microangiopathies : a descriptive systematic review.

BMC ophthalmology
2025

Acute oxalate nephropathy seven years post Roux-en-Y gastric bypass surgery without excessive vitamin C intake: a case report.

BMC geriatrics
2026

Severe hypertension with thrombotic microangiopathy: the need for pathogenically targeted treatments.

Kidney international
2025

TREX1-associated thrombotic microangiopathy: a tale of 2 mechanistically distinct domains.

Kidney international
2025

[Ultra-early administration of eculizumab in a child with atypical hemolytic uremic syndrome: a case report].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
2026

Early Echocardiographic and Serum Biomarkers Predict Thrombotic Microangiopathy, Endotheliopathy, and Survival After Pediatric Hematopoietic Stem Cell Transplant.

Transplantation and cellular therapy
2025

Case Report: A rare complication of non-hypertensive HELLP syndrome-microangiopathic coagulation activation leading to postpartum DIC with acute kidney injury.

Frontiers in medicine
2025

Multidisciplinary management of concurrent postpartum eclampsia, HELLP syndrome, ruptured hepatic subcapsular hematoma, and atypical hemolytic uremic syndrome: a case report.

BMC pregnancy and childbirth
2026

Acute Toxicities and Early Outcomes of Tandem Autologous Stem Cell Transplantation in Pediatric High-Risk Neuroblastoma: A Multicenter Study.

Transplantation and cellular therapy
2026

Malignant Hypertension-Induced Thrombotic Microangiopathy Mimicking Atypical Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura.

JACC. Case reports
2025

Platelet Count of Zero: A Case of Vancomycin-Induced Immune Thrombocytopenia.

Cureus
2026

Primary antiphospholipid syndrome and the kidney: biopsy-proven insights from two referral Italian centres.

Rheumatology (Oxford, England)
2025

Transient Haemolytic Anaemia and Thrombocytopenia in a Healthy Young Adult Following Influenza a Infection.

European journal of case reports in internal medicine
2025

Chronic Glomerular Thrombotic Microangiopathy in a 72-Year-Old Patient with B-Cell Chronic Lymphocytic Leukemia and IgG Lambda Paraprotein.

International journal of molecular sciences
2025

Spectrum and outcomes of thrombotic microangiopathies in nephrology: a 17-year cohort from a North African center.

BMC nephrology
2025

Protocol for the Isolation and Analysis of Extracellular Vesicles From Peripheral Blood: Red Cell, Endothelial, and Platelet-Derived Extracellular Vesicles.

Bio-protocol
2026

Safety and Effectiveness of Emicizumab in People With Haemophilia A Enrolled in the ATHN 7 Haemophilia Natural History Study.

Haemophilia : the official journal of the World Federation of Hemophilia
2025

Exploring the Relationship Between Thrombotic Microangiopathy and Immune Checkpoint Inhibitors: A Focus on Mechanistic Insights.

American journal of therapeutics
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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Microangiopatia trombótica

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. [Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].
    Zhonghua er ke za zhi = Chinese journal of pediatrics· 2026· PMID 41834207mais citado
  2. Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.
    JAMA network open· 2026· PMID 41805954mais citado
  3. Role of extracorporeal therapy in poisoning - A case based narrative.
    International journal of critical illness and injury science· 2026· PMID 41798046mais citado
  4. Presentation of HIV-Associated Thrombotic Microangiopathy and Response to Therapeutic Plasma Exchange: A 10-year Retrospective Single-Centre Cohort Study.
    Health science reports· 2026· PMID 41788639mais citado
  5. Reinventing the wheel in ischemic stroke: Novel aspects in ADAMTS13 and von Willebrand axis.
    Blood reviews· 2026· PMID 41760431mais citado
  6. Chemotherapy-Induced Thrombotic Microangiopathy in a Pediatric Patient With a Single Functioning Kidney: A Case Report and Review of the Literature.
    Cureus· 2026· PMID 41994719recente
  7. Acquired Thrombotic Thrombocytopenic Purpura with a Poor Response to Treatment can be an Initial Manifestation of Occult Signet Ring Cell Carcinoma and other Malignancies.
    Eur J Case Rep Intern Med· 2026· PMID 41988548recente
  8. Case Report: COVID-19 unmasks factor H mutation-driven hemolytic uremic syndrome in a previously undiagnosed septuagenarian kidney transplant recipient.
    Front Med (Lausanne)· 2026· PMID 41987803recente
  9. Bartonella henselae infection associated with a multisystemic thrombo-inflammatory syndrome in an immunocompetent adult.
    Infection· 2026· PMID 41986819recente
  10. A Case of Urethral Carcinoma With Cardiac Metastasis and Pulmonary Tumor Thrombotic Microangiopathy Confirmed by Autopsy.
    Cureus· 2026· PMID 41982566recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:93573(Orphanet)
  2. MONDO:0019737(MONDO)
  3. GARD:19227(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q3312044(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Microangiopatia trombótica
Compêndio · Raras BR

Microangiopatia trombótica

ORPHA:93573 · MONDO:0019737
CID-10
M31.1 · Microangiopatia trombótica
Medicamentos
13 registrados
MedGen
UMLS
C2717961
EuropePMC
Wikidata
Papers 10a
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