É uma doença autoimune em que a quantidade de plaquetas no sangue diminui porque o próprio sistema de defesa do corpo (o sistema imunológico) as ataca e destrói. O diagnóstico de PTI é feito por exclusão, ou seja, é confirmado apenas depois que outras causas são descartadas. Além disso, a doença pode ter diferentes origens.
Introdução
O que você precisa saber de cara
É uma doença autoimune em que a quantidade de plaquetas no sangue diminui porque o próprio sistema de defesa do corpo (o sistema imunológico) as ataca e destrói. O diagnóstico de PTI é feito por exclusão, ou seja, é confirmado apenas depois que outras causas são descartadas. Além disso, a doença pode ter diferentes origens.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 23 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
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
CytoplasmCell membrane
Medicamentos aprovados (FDA)
3 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
12 variantes patogênicas registradas no ClinVar.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Trombocitopenia imune
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Ensaios clínicos abertos e novidades científicas recentes
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 4.359
Development and Validation of a Novel Thrombosis Prediction Model for Adult Immune Thrombocytopenia (ITP-THROMBO).
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by bleeding, yet paradoxically, it can also predispose patients to thrombotic events; therefore, investigating high-risk factors for thrombosis in ITP patients and developing a predictive model is essential. A total of 1112 patients were diagnosed with ITP between January 2018 and December 2023. Excluding 216 patients under the age of 18, a total of 896 adult ITP patients were included in this study (of whom 101 developed thrombosis). Patients were randomly allocated to the training set (n=628) and validation set (n=268) in a 7:3 ratio. Atrial fibrillation, peripheral vascular disease, venous thromboembolism history, pneumonia < 1 month, cerebrovascular events history, and D-dimer levels were identified as predictive factors for thrombosis in ITP patients. The six factors formed 57 unique combinations, providing robust predictive power for thrombosis in ITP under different clinical scenarios. In the training set, the area under the curve (AUC) for the nomogram was 0.656 (95% CI: 0.578-0.735) to 0.931 (95% CI: 0.902-0.960) and the AUC in the validation set was 0.539 (95% CI: 0.425-0.653) to 0.893 (95% CI: 0.828-0.957). The calibration curve demonstrated good concordance between the model's predicted probabilities and actual observed probabilities, and the decision curve analysis indicated that the model had significant clinical utility. This cohort study developed a simple and practical predictive model (ITP-THROMBO) for estimating thrombosis risk in ITP patients; this model facilitates rapid identification of ITP patients at high thrombotic risk, enabling timely decision-making support for personalized treatment planning.
Managing heavy menstrual bleeding in adolescents with bleeding disorders: Outcomes from a pragmatic LMIC approach.
Heavy menstrual bleeding (HMB) a common manifestation of bleeding disorders in adolescents contributes to anemia, transfusions, and impaired quality-of-life (QOL). Evidence supporting practical management strategies in resource-limited settings remains limited. This observational study in adolescents with confirmed bleeding disorders with HMB at a tertiary hemophilia treatment centre evaluated menstrual blood loss and QOL after a standard regimen of progesterone, tranexamic acid and iron; blood products or clotting factor concentrates were reserved for refractory bleeding. Twenty adolescents were included (2020-2025); von Willebrand disease (n = 11), chronic immune thrombocytopenia (n = 4), congenital aplastic anemia (n = 3), afibrinogenemia (n = 1), and factor VII deficiency (n = 1). Median PBAC score at presentation was 300 (range 190-500). Eighteen patients (90%) had anemia and prior transfusion exposure. After therapy, median PBAC declined to 100 (range 60-150). Hemoglobin improved steadily and no patient required further red cell transfusion during a median follow-up of four years. Hemostatic products were required only in two patients while on this regimen. Quality-of-life scores improved across physical, emotional, and social domains. A low-cost regimen combining progesterone, antifibrinolytic therapy, and iron effectively controls HMB, corrects anemia, reduces transfusion and factor utilization, and improves QOL in adolescents with bleeding disorders in resource-constrained settings.
Towards achieving immune reset in autoimmune cytopenias: Insights from clinical experiences with B cell depleting therapies.
Autoimmune cytopenias (AICs) arise from pathogenic autoantibody-mediated destruction of blood cells. Current treatments often fail to achieve durable remission, necessitating long-term treatment including immunosuppression and exposure to treatment-related toxicities. Insights into B cell biology demonstrate the central role of autoreactive B cells and plasma cells in sustaining disease activity and relapse, providing a rationale to achieve sustained, treatment-free remissions through "immune reset". This review summarizes the current understanding of immune reset in AICs and examines clinical data for depletion strategies targeting distinct stages of B cell maturation. We compare therapeutic modalities across monoclonal antibodies, bispecific T cell engagers (TCE), and chimeric antigen receptor (CAR) T cells. The depth and breadth of depletion, kinetics of immune reconstitution, and treatment-related risks are critical determinants of long-term outcome potential and individualized risk-benefit assessment. Collectively, these advances support a potential paradigm shift from chronic immunosuppression towards durable remissions off therapy with time-limited therapeutic interventions.
IRE1α regulates macrophage phagocytosis in immune thrombocytopenia through NR1D1 mRNA decay and lysosomal biogenesis.
Macrophage phagocytosis is essential for immune homeostasis but must be tightly constrained to prevent pathological tissue damage. How cellular stress pathways enforce phagocytic homeostasis remains incompletely understood. Here, we show that phagocytosis selectively activates the endoplasmic reticulum stress sensor IRE1α in macrophages, which functions as a negative regulator of lysosome-driven phagocytic amplification. Using myeloid-specific IRE1α-deficient mice and pharmacological inhibition, we demonstrate that loss of IRE1α RNase activity leads to excessive phagocytosis through unchecked lysosomal biogenesis. Mechanistically, phagocytosis-activated IRE1α directly degrades Nr1d1 mRNA via regulated IRE1α-dependent decay (RIDD), thereby restraining NR1D1-driven lysosomal expansion. Disruption of this IRE1α-NR1D1 axis exacerbates macrophage-mediated platelet clearance and accelerates disease progression of immune thrombocytopenia (ITP). Reduced ERN1 expression and IRE1α activity are observed in monocytes from patients with ITP. Pharmacological inhibition of NR1D1 or lysosomal activity rescues thrombocytopenia. Together, these findings establish the IRE1α-NR1D1-lysosome axis as a therapeutically actionable pathway in phagocytosis-driven diseases.
Evaluation of the Real-World Efficacy of Hetrombopag for the Treatment of Thrombocytopenia Postallogeneic Hematopoietic Stem Cell Transplantation.
Persistent thrombocytopenia (PT), comprising delayed platelet engraftment (DPE) and secondary failure of platelet recovery (SFPR), is a severe complication following allogeneic hematopoietic stem cell transplantation (allo-HSCT), occurring in up to 70% of recipients. PT is strongly associated with life-threatening hemorrhage, increased nonrelapse mortality, and reduced overall survival (OS). Current therapeutic options, including recombinant human thrombopoietin (rhTPO), CD34+ cell boosts, and mesenchymal stromal cell infusion, are limited by variable efficacy and procedural complexity. TPO receptor agonists (RAs) have shown promise in promoting megakaryopoiesis, yet their efficacy in the post-transplant setting remains heterogeneous. Hetrombopag, a novel oral TPO-RA with enhanced receptor affinity and a favorable safety profile, has demonstrated efficacy in immune thrombocytopenia and aplastic anemia, but its real-world utility in allo-HSCT-associated PT remains unexplored. This study aimed to evaluate the real-world efficacy and safety of hetrombopag in patients with PT following allo-HSCT, in order to improve outcomes for this high-risk population. This retrospective observational study enrolled 33 adult patients with PT (7 DPE, 26 SFPR) after allo-HSCT between June 2022 and April 2024. All patients received oral hetrombopag starting at 5 mg/d, with escalation to 7.5 mg/d if no response was observed within 1 wk. Treatment continued until platelet recovery, adverse events, or disease progression. The primary endpoint was the overall response rate (ORR), defined as sustained platelet recovery to ≥20 × 10⁹/L for ≥7 d without transfusion. Secondary endpoints included OS, relapse, graft-versus-host disease, and safety. Statistical analyses included Kaplan-Meier survival analysis, Cox regression, and nonparametric tests for variable comparisons. The median platelet count before treatment was 14 × 10⁹/L. Hetrombopag was initiated at a median of 56 d post-transplant, with a median treatment duration of 82 d. The ORR was 81.8% (27/33), with 100% response in DPE and 76.9% in SFPR subgroups. Among responders, median platelet counts increased from 14 to 80 × 10⁹/L, and megakaryocyte counts rose from 12 to 58 per smear. Notably, 6 of 7 patients with absent megakaryocytes at baseline responded to treatment. Of 11 patients previously unresponsive to rhTPO, 10 responded to hetrombopag. No significant predictors of complete response were identified in univariate analysis. Safety analysis revealed mild liver enzyme elevation in 4 patients (12.1%), with no grade 3 to 4 toxicities or treatment discontinuations. Hetrombopag demonstrates high efficacy and a favorable safety profile in the management of PT after allo-HSCT, including in patients with poor megakaryocyte reserves or prior rhTPO failure. These real-world findings support its use as a promising therapeutic option in this high-risk population. Further prospective, randomized studies are needed to validate these results and establish predictive biomarkers for optimal patient selection.
Publicações recentes
Chimeric antigen receptor T cells for autoimmune diseases, in particular immune thrombocytopenia.
Isolated Dermal Arteriolar Thrombosis in a Pediatric Patient: A Rare Complication During Eltrombopag Therapy for Chronic Immune Thrombocytopenia.
MicroRNAs as potential architects of immune dysregulation and megakaryocytic failure in immune thrombocytopenia.
[Refractory systemic lupus erythematosus-associated thrombocytopenia treated with avatrombopag: A case report].
Alcohol-Induced Acute Pancreatitis Complicated by Severe Direct Antiglobulin Test (DAT)-Negative Hemolytic Anemia and Thrombocytopenia: A Suspected Evans Syndrome-Like Immune Cytopenia.
📚 EuropePMC4.283 artigos no totalmostrando 198
The role of T Cell Immunoglobulin Mucin Domain-3 in children with immune thrombocytopenia.
BMC pediatricsStructural Mass-Spectrometric Description of Immune Complexes in Vaccine-Induced Immune Thrombocytopenia and Thrombosis.
Journal of the American Chemical SocietyThrombopoietin receptor agonists in immune thrombocytopenia: a comparative review of mechanisms, efficacy, and the future of personalized management.
Expert opinion on pharmacotherapyPediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center.
Pediatric reportsSteroid-Resistant Immune Thrombocytopenia With Severe Bleeding Successfully Managed Using Eltrombopag and Mycophenolate in a 67-Year-Old Male.
CureusA complete blood count-based machine learning model for rapid differentiation of aplastic anemia, immune thrombocytopenia, and myelodysplastic syndromes in routine clinical practice.
Practical laboratory medicineDevelopment and Validation of a Novel Thrombosis Prediction Model for Adult Immune Thrombocytopenia (ITP-THROMBO).
Journal of blood medicineManaging heavy menstrual bleeding in adolescents with bleeding disorders: Outcomes from a pragmatic LMIC approach.
Blood cells, molecules & diseasesReal-World Safety and Efficacy of Avatrombopag in Adults with Immune Thrombocytopenia: A Systematic Review and Meta-Analysis.
European journal of haematologyExploring exosome-related genes as candidate biomarkers in primary immune thrombocytopenia through transcriptomics and preliminary experimental validation.
Scientific reportsAssociation Between Bleeding Severity and Quality of Life in Adults with Immune Thrombocytopenia.
Juntendo medical journalPrimary Sjögren's syndrome-associated immune thrombocytopenia: from pathogenesis to treatment.
Frontiers in immunologyThe glycan fingerprint in immune thrombocytopenia.
Research and practice in thrombosis and haemostasisAnti-CD19 CAR T cell therapy for refractory SLE-ITP.
Med (New York, N.Y.)Bernard Soulier Syndrome Misdiagnosed and Treated as Immune Thrombocytopenia Purpura: A Case Report.
CureusSolving the mystery of vaccine-induced immune thrombocytopenia and thrombosis.
Nature reviews. CardiologyTowards achieving immune reset in autoimmune cytopenias: Insights from clinical experiences with B cell depleting therapies.
Blood reviewsMonoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
Journal of thrombosis and haemostasis : JTHHematologic adverse events following COVID-19 vaccination and revaccination in persons aged 12 and above: A Canadian Immunization Research Network study.
Human vaccines & immunotherapeuticsEfficacy and safety of TPO-RA combined with sirolimus in the treatment of relapsed immune thrombocytopenia.
Hematology (Amsterdam, Netherlands)[Bleeding characteristics in pregnant patients with immune thrombocytopenia and severe thrombocytopenia].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi[Construction and evaluation of a thrombosis risk prediction model in patients with primary immune thrombocytopenia].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiA Diagnostic Challenge in a Child With Recurrent Thrombocytopenia: Cyclic Thrombocytopenia Associated With Immune Dysregulation.
Pediatric blood & cancerPrevalence and associated factors of sexual dysfunction in female patients with immune thrombocytopenia: a longitudinal follow-up study.
BMC women's healthThe impact of body mass index on treatment response to high-dose dexamethasone in adult primary immune thrombocytopenia patients: A two-center retrospective study.
Internal and emergency medicineIRE1α regulates macrophage phagocytosis in immune thrombocytopenia through NR1D1 mRNA decay and lysosomal biogenesis.
Cell reportsClinical Impact of Eltrombopag-Associated Iron Chelation in Adults with Immune Thrombocytopenia: A Multicenter Real-World Study.
Mediterranean journal of hematology and infectious diseasesThe PI3K-AKT pathway mediates the imbalance of bone marrow macrophage polarization in patients with immune thrombocytopenia.
Journal of translational medicineNo increased risk of acute myeloid leukemia in adults with primary immune thrombocytopenia treated with thrombopoietin receptor agonists: a French nationwide population-based study.
HaematologicaImmature Platelet Fraction and Absolute Immature Platelet Count as Diagnostic Markers in Pediatric Immune Thrombocytopenia.
International journal of laboratory hematologyShort- and long-term outcome of neonates with thrombocytopenia from maternal immune thrombocytopenia.
British journal of haematologyMyeloid-Derived Suppressor Cells: Immunoregulatory Roles and Therapeutic Prospects in Immune-Mediated Hematological Disorders.
Journal of immunology researchLonger time to response in hemato-oncological patients with immune thrombocytopenia (ITP): a pilot study.
Expert review of hematologyLongitudinal clinical and preclinical studies identify hetrombopag as a potent chelator for systemic iron overload.
HemaSphereDapsone response in immune thrombocytopenic purpura is associated with dapsone dose and mediated through reduction in haemoglobin: A longitudinal data analysis of 58 adult and paediatric ITP patients demonstrates efficacy and safety.
British journal of haematologyCost-effectiveness of hetrombopag, eltrombopag, and avatrombopag for chronic immune thrombocytopenia in China: a cost-utility analysis.
Frontiers in public healthTrends of Treatments for Pediatric Patients with Immune Thrombocytopenia in Japan.
Annals of clinical epidemiologyFrom Hemolysis to Lupus: A Case of Evans Syndrome Revealing Systemic Autoimmunity.
Clinical case reportsLack of association between vaccine-induced immune thrombocytopenia and thrombosis and HLA loci in a large cohort.
British journal of haematologyEvaluation of the Real-World Efficacy of Hetrombopag for the Treatment of Thrombocytopenia Postallogeneic Hematopoietic Stem Cell Transplantation.
Transplantation and cellular therapyEvaluation of platelet surface-associated immunoglobulin positivity and its association with hematologic findings and vector-borne pathogens in thrombocytopenic dogs.
Journal of veterinary internal medicineSevere Persistent Neonatal Immune Thrombocytopenia Despite Optimized Maternal Chronic Immune Thrombocytopenia (ITP) Management.
CureusSustained response off-treatment and thrombotic events in patients with immune thrombocytopenia treated with fostamatinib: a systematic review and meta-analysis.
HaematologicaEfficacy and safety of immunosuppressive therapy in connective tissue disease-related immune thrombocytopenia: a systematic review and meta-analysis.
Therapeutic advances in hematologySevere Transfusion-refractory Cytopenia Mimicking Bone Marrow Failure in a Patient Living with HIV after Antiretroviral Therapy Interruption.
Internal medicine (Tokyo, Japan)[Efficacy of fostamatinib add-on for TPO receptor agonist-refractory immune thrombocytopenia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyBlinatumomab in Combined Immune Thrombocytopenia and Antiphospholipid Syndrome.
The New England journal of medicineNovel use of telitacicept in primary immune thrombocytopenia: a case report.
BMC pediatricsCDK8/CDK19 inhibition restores T-cell homeostasis in primary immune thrombocytopenia.
BloodOptimizing pediatric ITP therapy: a real-world study on plasma concentration-guided eltrombopag dosing.
Annals of hematologyThromboembolic adverse events associated with TPO-RA in ITP treatment: a pharmacovigilance analysis of the FDA Adverse Event Reporting System.
Frontiers in immunologyPropofol and/or etomidate-induced immune thrombocytopenia: A case report.
Hematology (Amsterdam, Netherlands)A Clinician Perspective for a Personalized Approach to Management of Chronic Immune Thrombocytopenia with Targeted Therapies Alone or in Combination.
Journal of clinical medicineCASP8 and CASP3 mRNA Expression in Autoimmune Lymphoproliferative Syndrome (ALPS) and Chronic Immune Thrombocytopenia (ITP).
GenesTreatment outcomes and prognostic indicators of primary immune thrombocytopenia in 31 cats: a multicenter retrospective study (2000-2023).
Journal of veterinary internal medicineLong-term efficacy and safety of low-dose rituximab in immune thrombocytopenia: a multicentre, prospective, open-label, randomised controlled trial.
Annals of hematologyMultifactorial thrombotic risk in Evans syndrome: a case report and review of the literature.
Thrombosis journalCongenital Amegakaryocytic Thrombocytopenia Presenting With Features of Immune Thrombocytopenia: A Case Report.
CureusClinical and Genetic Characterization of 269 Patients With Suspected Inherited Platelet Disorders: The Padua Monocentric Experience.
International journal of laboratory hematologyLife-threatening intraparenchymal hematoma due to immune thrombocytopenic purpura: illustrative case.
Journal of neurosurgery. Case lessonsCharacteristics and Outcomes of Patients with Hodgkin Lymphoma with Paraneoplastic Manifestations.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionSuccessful Treatment of Multirefractory Immune Thrombocytopenia in Rubinstein-Taybi Syndrome With Combined Rituximab and Eltrombopag.
Pediatric blood & cancerA Case Report of Thrombocytopenia Caused by a May-Hegglin Anomaly in a Young Saudi Female.
Journal of investigative medicine high impact case reportsEfficacy and safety of perioperative thrombopoietin receptor agonists in patients with immune thrombocytopenia and thrombocytopenia secondary to chronic liver disease undergoing elective procedures: A systematic review and meta-analysis.
Journal of thrombosis and thrombolysisA Potential Case of Tetanus-Diphtheria Vaccine-Induced Immune Thrombocytopenia.
CureusEffects of Fibrinogen Levels on Thromboelastography-Derived Clot Strength in Pregnant Women With or Without Immune Thrombocytopenia.
Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/HemostasisPlatelet Satellitism Causing Apparent Loss of Response to Eltrombopag in a Patient With Immune Thrombocytopenia.
International journal of laboratory hematologySevere Immune Thrombocytopenic Purpura Associated With Acute Epstein-Barr Virus Infection: A Case Report.
CureusA case of immune thrombocytopenia aggravated by therapy for hypercortisolism in adrenocortical carcinoma.
Therapeutic advances in endocrinology and metabolismNingxue Shengban decoction containing serum alleviates immune thrombocytopenia by modulating CD4+T cells balance via BMSCs-Exo-miR-199a-5p.
Stem cell research & therapyRefractory Primary Immune Thrombocytopenia With Bleeding and Thrombosis: A Case Report.
The American journal of case reportsIdentification of key biomarkers for initial corticosteroid resistance in newly diagnosed primary immune thrombocytopenia.
Experimental hematologyTranslational immunothrombosis in autoimmune Heparin-Induced thrombocytopenia: targeting the FcγRIIa-Syk-BTK and complement pathways.
Clinical and experimental medicineCharacterization of monoclonal and patient-derived antiplatelet factor 4 antibodies in platelet factor 4 and platelet factor 4/polyanion chemiluminescence assays.
Journal of thrombosis and haemostasis : JTHImmunopathology of immune thrombocytopenia.
Journal of thrombosis and haemostasis : JTHAdenoviral Inciting Antigen and Somatic Hypermutation in VITT.
The New England journal of medicineThe Genetic Link Between Primary Immune Thrombocytopenia and Depression/Anxiety Disorders: A Two-Sample Mendelian Randomization Study.
Journal of clinical laboratory analysisWatermelon Duodenum (Duodenal Bulb Vascular Ectasia) in a Patient With Hepatocellular Carcinoma: A Rare Cause of Upper Gastrointestinal Bleeding.
CureusUnintended First-Trimester Exposure to Two Distinct Thrombopoietin Receptor Agonists in Consecutive Pregnancies Without Maternal or Fetal Harm.
Turkish journal of haematology : official journal of Turkish Society of HaematologySuccessful Management of Relapsed Severe Immune Thrombocytopenia Using Avatrombopag: A Case Report.
Case reports in hematologyPerioperative Management of Immune Thrombocytopenic Purpura in Cardiac Surgery: A Systematic Review of the Literature in Urgent and Elective Settings.
CureusAdvances in our understanding of anti-PF4 related immunothrombosis.
Frontiers in immunologyMulticenter initiative to standardize management of pediatric immune thrombocytopenia improves adherence to guidelines.
Blood advancesPulmonary Embolism and Myocardial Infarction With Non-obstructive Coronary Arteries in Immune Thrombocytopenia: Unmasking Underlying Antiphospholipid Syndrome.
CureusDrug-Induced Immune Thrombocytopenia Secondary to Trimethoprim-Sulfamethoxazole.
Case reports in infectious diseasesNingxue shengban decoction regulates T-cell immune balance in immune thrombocytopenia via the bone marrow hematopoietic microenvironment.
Computational biology and chemistryGuideline on the emergency management of critical bleeding in patients with immune thrombocytopenia.
Blood advancesPredicting Development of Pediatric Chronic Immune Thrombocytopenia at Disease Onset Using a Statistical Risk Model.
BloodIntravenous Thiamine-Induced Thrombocytopenia in a Patient With Chronic Adrenal Insufficiency.
Journal of medical casesInflammatory cytokines, inflammasomes, and neutrophil extracellular traps in primary immune thrombocytopenia pathogenesis.
Blood vessels, thrombosis & hemostasisUnmasking Wiskott-Aldrich Syndrome in Adulthood in a Case of Long-Standing Bleeding, Infections, and Steroid-Induced Morbidity.
CureusSuccessful Treatment of Oxaliplatin-Induced Immune Thrombocytopenia in a Schizophrenic Patient With Contraindication to Steroids Using Cyclosporine Plus Eltrombopag: A Case Report.
Case reports in medicineFc-dependent and -independent Platelet Clearance Caused by Anti-CD36 IgG1 and IgG2 Subclasses.
Thrombosis and haemostasisDe Novo MYH9-Related Macrothrombocytopenia in a Toddler: Insights From Platelet Mass Index.
British journal of hospital medicine (London, England : 2005)Blinatumomab-induced remission of refractory immune thrombocytopenia in pediatric acute lymphoblastic leukemia: a case report.
HaematologicaMethylprednisolone-induced immune thrombocytopenia in a patient with severe bullous pemphigoid.
European journal of dermatology : EJDNo increased risk of hepatic enzyme abnormalities with non-peptidic thrombopoietin receptor agonists in immune thrombocytopenia: A meta-analysis of randomized controlled trials.
Experimental and therapeutic medicineMiliary Tuberculosis With Immune Thrombocytopenia in 50-Year-Old Ethiopian Woman: A Case Report and Brief Review of Literature.
Clinical case reportsImmune thrombocytopenia: contemporary pathophysiology, treatment gaps, and the role of novel mechanisms in patient-centered care.
The American journal of managed careMultisystem Complications in Postpartum-Onset Evans Syndrome: A Case Report.
CureusOriginal Versus Generic Eltrombopag in Patients with Immune Thrombocytopenia: A Prospective Multi-Center Experience on Efficacy and Safety.
Journal of clinical medicineAn integrative analysis of genetic factors reveals dysregulation of cytokines, immune signaling, and T cell activity as the underlying immune mechanisms in autoimmune immune thrombocytopenia.
Thrombosis journalPatient and Physician Experiences in Immune Thrombocytopenia.
Advances in therapyProperties of FDA-approved small molecule protein kinase inhibitors: A 2026 update.
Pharmacological researchBCMA-CD3 T-cell engager as salvage therapy for multirefractory primary immune thrombocytopenia.
Lancet (London, England)Comparison of the efficacy of thalidomide combined with low-dose rituximab and low-dose rituximab alone in the treatment of steroid-resistant/refractory adult patients with primary immune thrombocytopenia: an open-label trial.
Frontiers in medicineAmegakaryocytic thrombocytopenia in a dog: Diagnostic challenges and therapeutic considerations.
The Canadian veterinary journal = La revue veterinaire canadienneHistiocytic Sarcoma Treated With Autologous Stem Cell Transplantation: A Case Report and Literature Review of the Role of Autologous and Allogenic Stem Cell Transplantation.
The American journal of case reportsImmune-Mediated Multilineage Cytopenias in Thymoma: A Retrospective Case Series.
Thoracic cancerCorrigendum to "Clinical Efficacy of Guishao Shengxue Decoction in the Treatment of Pediatric Immune Thrombocytopenia: A Randomized Controlled Trial" [EXPLORE 21 (2025) 103277].
Explore (New York, N.Y.)[From heparin-induced thrombocytopenia to the emergence of VITT like syndromes].
La Revue du praticienCharacteristics of adverse events and clinical risks of intravenous immunoglobulin: a pharmacovigilance study based on FDA Adverse Event Reporting System (FAERS).
Frontiers in medicineImmune thrombocytopenia in patients with connective tissue disease.
Clinical rheumatologyImmune Thrombocytopenia Successfully Controlled by Dissection of an Enlarged Mediastinal Lymph Node Metastasis from Squamous Cell Carcinoma of Unknown Primary: A Case Report.
Surgical case reportsImmune repertoire profiling in immune thrombocytopenia: A path towards biomarkers, for The British Journal of Haematology.
British journal of haematologyElevated IGF1R and abnormal methylated IGF1R in immune thrombocytopenia.
Annals of hematologyLong-term efficacy and safety of hetrombopag in paediatric patients with immune thrombocytopenia.
British journal of haematology[A Case Study of Severe Myelosuppression Induced by Adjuvant Chemotherapy in a Breast Cancer Patient with Concurrent Immune Thrombocytopenia(ITP)].
Gan to kagaku ryoho. Cancer & chemotherapyEffectiveness of Rituximab, Belimumab, and Thrombopoietin Receptor Agonists in Refractory Immune Thrombocytopenia Associated With Systemic Lupus Erythematosus: A Systematic Review and Meta-Analysis.
CureusThe interleukin-1 receptor antagonist gene VNTR polymorphism confers a genetic contribution to the risk of immune thrombocytopenia purpura: A systematic review and meta-analysis.
CytokinePredicting Chronicity in Children and Adolescents With Newly Diagnosed Immune Thrombocytopenia at the Timepoint of Diagnosis Using Machine Learning-Based Approaches.
Pediatric blood & cancerPediatric Evans Syndrome Diagnostic Evaluation Patterns: Survey Results From the Pediatric ITP Consortium of North America.
Pediatric blood & cancerIntegrating "Yang transforming Qi and Yin constituting the body" with immune regulation: an evidence synthesis of multidimensional traditional chinese medicine therapy for immune thrombocytopenia.
Chinese medicineReal-World Effectiveness and Safety of Fostamatinib in Difficult-to-Treat Immune Thrombocytopenia Patients. A Prospective, Multicenter Registry in France.
American journal of hematologyIncidence, Characteristics, and Management of Venous Thrombosis in Adult Patients With Immune Thrombocytopenia: Results From the Multicenter, Prospective Registry CARMEN-France.
American journal of hematology[The Mechanism of Echinococcus Granulosus Sensu Stricto Antigen B to Protect Immune Thrombocytopenia Mouse Model by Influencing Autophagy].
Zhongguo shi yan xue ye xue za zhiEfficacy and safety of eltrombopag in combination with cyclosporine A for the treatment of adult refractory primary immune thrombocytopenia: a phase II, multicenter, single-arm, prospective study.
Clinical and experimental medicineIncidence, Risk Factors, and Treatment of Autoimmune Cytopenia Following Pediatric Allogeneic Hematopoietic Stem Cell Transplantation.
Journal of transplantationRilzabrutinib in immune thrombocytopenia: a targeted, patient-centered alternative to conventional therapies.
Annals of medicine and surgery (2012)Efficacy and safety of eltrombopag in combination with dexamethasone as first-line treatment in adult patients with newly diagnosed immune thrombocytopenia.
British journal of haematologyPhysiological evaluation of the emotional regulation of patients with hereditary angioedema.
BioPsychoSocial medicineTNFAIP3 Gene Polymorphisms and the Risk of Thrombocytopenia: A Cross-Sectional Study in Iranian Population.
Health science reportsSafety and efficacy of spleen tyrosine kinase (Syk) inhibitors in the treatment of adults with persistent and chronic immune thrombocytopenia (ITP): A systematic review and meta-analysis of randomised clinical trials.
British journal of haematology[Clinical characteristics and prognosis of primary immune thrombocytopenia complicated with diffuse alveolar hemorrhage].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi[Chinese guideline on the diagnosis and treatment of adult primary immune thrombocytopenia (2025)].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiHigh pretreatment peripheral blood T-cell receptor clonality as a predictor of prolonged response in immune thrombocytopenia.
British journal of haematologyAssessment of IgG N-Glycan patterns in adult patients with immune thrombocytopenia and healthy individuals.
Glycoconjugate journalManagement of Hemorrhagic Risk in Refractory Immune Thrombocytopenia Complicating a Dichorionic Diamniotic Twin Pregnancy.
Seminars in thrombosis and hemostasisCombining an immunomodulatory drug with a TPO-RA to treat multirefractory ITP patients: The Spanish ITP Group experience.
British journal of haematologyImmune Thrombocytopenia and Type 1 von Willebrand Disease in a Patient With a Femoral Fracture: A Case Report.
CureusAvatrombopag in immune thrombocytopenia and beyond: Current evidence and emerging perspectives.
Blood reviewsMechanistic insights into PD-1 inhibitor-associated immune thrombocytopenia: a case report integrating longitudinal transcriptomic profiling.
Journal for immunotherapy of cancerAdverse events post-BNT162b2 vaccination in Japanese children.
VaccineGrade 4 thrombocytopenia with PD-1 inhibitors following progression of PD-L1 inhibitor therapy in small cell lung cancer: a case report and literature review.
BMC geriatricsEfgartigimod for Generalized Myasthenia Gravis and Beyond: A Narrative Review of Its Pharmacological Profile, Clinical Utility, and Expanding Applications.
BiomedicinesSpleen and Bruton's tyrosine kinase inhibitors for the management of adult immune thrombocytopenia: A systematic review and meta-analysis of randomized controlled trials.
Clinical hematology internationalRecurrent endometrial carcinoma with immune-mediated thrombocytopenia following durvalumab: A case report.
Case reports in women's healthThe roles and clinical applications of mesenchymal stem cells and their exosomes in hematologic diseases.
Stem cell research & therapyComparison of mean platelet component and mean platelet mass in immune thrombocytopenia versus hypoproductive thrombocytopenias.
Clinical biochemistryDouble Trouble: The First Reported Case of Evans Syndrome Following RSV Vaccination.
Hematology reportsManagement of Immune Thrombocytopenia via Splenectomy in a Full-Term Pregnant Woman: A Case Report.
International medical case reports journalInfluence of Anxiety Level and Degree of Alexithymia on Quality of Life in Adult Patients With Primary Immune Thrombocytopenia.
Actas espanolas de psiquiatriaNew drug treats chronic immune thrombocytopenia.
The American journal of nursingImmune thrombocytopenia triggered by dostarlimab in a patient with endometrial carcinoma: first reported case.
PlateletsWhat We Miss in Patients With Persistent Thrombocytopenia During the Dengue Season.
CureusIncidence of systemic lupus erythematosus in patients with immune thrombocytopenia: A nationwide study.
British journal of haematologySevere relapse of Evans syndrome in an adult patient with treatment resistance and fatal outcome: A case report.
The Journal of international medical researchDiagnostic and prognostic values of the systemic immune-inflammation index in children with immune thrombocytopenia.
Expert review of hematologyThe efficacy and safety of Huaiqihuang Granule in the treatment of childhood chronic primary immune thrombocytopenia: a multi-centre, randomized, double-blind, placebo-controlled clinical study ().
Journal of ethnopharmacologyCSF1R modulates megakaryopoiesis by targeting RUNX1 in immune thrombocytopenia.
HaematologicaAvatrombopag for treating children and adolescents with immune thrombocytopenia: a plain language summary.
Expert review of hematologySevere Octreotide-Induced Immune Thrombocytopenia With Bone Marrow Findings Suggestive of Acquired Amegakaryocytic Thrombocytopenic Purpura in a Cirrhotic Woman: An Unusual Case Report.
CureusC-Reactive Protein Mediates the Association Between Rheumatoid Arthritis and Immune Thrombocytopenia: An Observational and Mendelian Randomisation Study.
Scandinavian journal of immunologyImmature platelet fraction and bone marrow findings in hematology.
Scientific reportsPredicting treatment-free remission after thrombopoietin receptor agonist therapy in immune thrombocytopenia: real-world outcomes from a Korean cohort.
Journal of thrombosis and haemostasis : JTHImmune Imbalance and Dynamic Characteristics of T Helper 17, Regulatory T, and Regulatory B Cells in Children With Persistent Immune Thrombocytopenia.
Pediatric blood & cancerA Case of Cerebral Venous Sinus Thrombosis Following Eltrombopag in Immune Thrombocytopenia.
CureusAn Umbrella Review of Meta-Analyses on the Efficacy and Safety of Eltrombopag in Immune Thrombocytopenia.
Health science reportsAntigen B from Echinococcus granulosus regulates autophagy-mediated macrophage polarization to alleviate immune thrombocytopenia.
Parasites & vectorsSplenic megakaryocyte density in immune thrombocytopenia: insights from a histologic analysis of splenectomy specimens.
International journal of clinical and experimental pathologyANA-Positive Primary Immune Thrombocytopenia: An Independent Clinical Entity-Potential Connective Tissue Diseases?
International journal of rheumatic diseasesAre there long-term effects of combination therapy for newly diagnosed adults with ITP?
British journal of haematologyEfficacy and safety of dapsone in adult immune thrombocytopenia: a systematic review and meta-analysis.
European journal of medical researchUntargeted Metabolomic Profiling Reveals Lipid Metabolism Dysregulation in Patients With Immune Thrombocytopenia.
Scandinavian journal of immunologyRituximab-first versus eltrombopag monotherapy in pediatric persistent/chronic immune refractory thrombocytopenia after first-line therapy: a prospective multicenter cohort study.
Research and practice in thrombosis and haemostasisFostamatinib (R788), a spleen tyrosine kinase inhibitor, sensitizes pancreatic cancer cells to oncolytic vesicular stomatitis virus.
Molecular therapy. OncologyBruton's tyrosine kinase inhibition in ITP: Wayrilz (rilzabrutinib) as a disease-modifying strategy.
Annals of medicine and surgery (2012)Differentiating pathogenic from bystander autoantibodies in immune thrombocytopenia using intact glycoprotein-deficient megakaryocytes.
HaematologicaHetrombopag for patients with persistent primary immune thrombocytopenia: a post hoc analysis of a multicenter, randomized phase Ⅲ trial.
Research and practice in thrombosis and haemostasisIptacopan for Immune Thrombocytopenia and Cold Agglutinin Disease: A Global Phase 2 Basket Clinical Trial.
American journal of hematologyIanalumab plus Eltrombopag in Immune Thrombocytopenia.
The New England journal of medicineLongitudinal plasma proteomics in romiplostim-treated patients with immune thrombocytopenia.
British journal of haematologyImmune thrombocytopenia in patients treated with immune checkpoint inhibitors.
BloodRilzabrutinib: First Approval.
DrugsCombination of anti-CD20 and hetrombopag in relapsed/refractory immune thrombocytopenia: a case series.
Frontiers in medicineImmunomodulatory Mechanisms and Therapeutic Potential of Vitamin D in Immune Thrombocytopenia.
Journal of immunology researchMultimethodological and multiscale investigation of the therapeutic mechanism of Qian Ji Sheng Xue Pian in treating primary immune thrombocytopenia.
HereditasRefractory ITP: revisiting definitions, diagnostics, and management paradigms.
Hematology. American Society of Hematology. Education ProgramA practical approach to immune thrombocytopenia in pregnancy.
Hematology. American Society of Hematology. Education ProgramComparison of Ideal vs. Actual Body Weight Dosing of Intravenous Immunoglobulins for Immune Thrombocytopenia: A Retrospective Analysis.
Advances in hematologyPlasma interleukin-12 levels as a potential biomarker of fatigue and quality of life in patients with primary immune thrombocytopenia.
Blood researchTargeting the CCL2-CCR2 axis with ursodeoxycholic acid attenuates monocyte migration and platelet clearance in immune thrombocytopenia.
European journal of pharmacologyWartime Occurrence of Severe Aplastic Anemia in Previously Healthy Military Service Members: A Case Series.
Military medicineAcquired platelet disorders.
Frontiers in medicineSevere Thrombocytopenia in Pregnancy: Etiology, Management, and Outcomes Across Platelet Count Categories.
Journal of clinical medicinePaediatric Hospitalised Immune Thrombocytopenia in Real-Life Recent Viral Infection Outbreaks: A Retrospective Study.
Life (Basel, Switzerland)Clinical and Molecular Spectrum of MYH9-Thrombocytopenia: Insights from a Single Centric Pediatric Cohort.
Children (Basel, Switzerland)Platelet Recovery and Coexisting Conditions in Pediatric Immune Thrombocytopenia: Insights from a Tertiary Care Study.
Children (Basel, Switzerland)Associações
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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.
- Development and Validation of a Novel Thrombosis Prediction Model for Adult Immune Thrombocytopenia (ITP-THROMBO).
- Managing heavy menstrual bleeding in adolescents with bleeding disorders: Outcomes from a pragmatic LMIC approach.
- Towards achieving immune reset in autoimmune cytopenias: Insights from clinical experiences with B cell depleting therapies.
- IRE1α regulates macrophage phagocytosis in immune thrombocytopenia through NR1D1 mRNA decay and lysosomal biogenesis.
- Evaluation of the Real-World Efficacy of Hetrombopag for the Treatment of Thrombocytopenia Postallogeneic Hematopoietic Stem Cell Transplantation.
- Chimeric antigen receptor T cells for autoimmune diseases, in particular immune thrombocytopenia.
- Isolated Dermal Arteriolar Thrombosis in a Pediatric Patient: A Rare Complication During Eltrombopag Therapy for Chronic Immune Thrombocytopenia.
- MicroRNAs as potential architects of immune dysregulation and megakaryocytic failure in immune thrombocytopenia.
- [Refractory systemic lupus erythematosus-associated thrombocytopenia treated with avatrombopag: A case report].
- Alcohol-Induced Acute Pancreatitis Complicated by Severe Direct Antiglobulin Test (DAT)-Negative Hemolytic Anemia and Thrombocytopenia: A Suspected Evans Syndrome-Like Immune Cytopenia.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:3002(Orphanet)
- OMIM OMIM:188030(OMIM)
- MONDO:0008558(MONDO)
- Purpura Trombocitopenica Idiopatica(PCDT · Ministério da Saúde)
- GARD:5194(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q638729(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
