Lúpus eritematoso disseminado, ou simplesmente lúpus, é uma doença autoimune em que o sistema imunitário ataca tecidos saudáveis em várias partes do corpo. A intensidade dos sintomas varia de pessoa para pessoa, podendo ser ligeiros ou graves. Os sintomas mais comuns são articulações dolorosas e inchadas, febre, dor torácica, perda de cabelo, úlceras na boca, aumento de volume dos gânglios linfáticos, fadiga e uma erupção cutânea vermelha, mais comum no rosto. Geralmente alternam-se períodos de exacerbação dos sintomas e períodos de remissão com poucos sintomas.
Introdução
O que você precisa saber de cara
Anemia hemolítica autoimune neonatal é uma condição rara onde anticorpos maternos atacam os glóbulos vermelhos do feto/recém-nascido, causando destruição e anemia. Geralmente associada a incompatibilidade ABO ou Rh, pode levar a icterícia e, em casos graves, insuficiência cardíaca.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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)
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Publicações mais relevantes
Autoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
Autoimmune hemolytic anemias comprise warm AIHA (wAIHA), due to IgG autoantibodies reacting at body temperatures, and cold agglutinin disease (CAD), due to IgM reacting in cold and strongly activating complement. The diagnosis, based on the direct antiglobulin test (DAT), is sometimes difficult since the DAT is performed with various methods with different sensitivity and specificity. Novel investigation includes DAT by flow-cytometry that helps the diagnosis of DAT-negative forms and in-deep bone marrow examination that aims to understand AIHA pathogenesis. Therapy is different in wAIHA and CAD: in the former, steroids represent the first-line, followed by rituximab, splenectomy and immunosuppressors, while in the latter rituximab is recommended frontline, followed by rituximab plus chemotherapy or the complement inhibitor sutimlimab. Plasmacell-directed therapies (bortezomib and daratumumab) may be administered in refractory patients. Several new therapies are under investigation, including inhibitors of Bruton's and spleen tyrosine kinases, neonatal Fc receptor, and cytokines, and bi-specific monoclonal antibodies.
Targeting the neonatal Fc receptor (FcRn) in hematologic conditions with a focus on warm autoimmune hemolytic anemia.
Warm autoimmune hemolytic anemia (wAIHA) is the most prevalent form of autoimmune hemolytic anemia, where in most patients extravascular hemolysis is driven by immunoglobulin G (IgG) autoantibodies, with or without complement activation. While standard-of-care treatment with corticosteroids provides a high initial response rate, relapses are frequent, and most patients require additional immunosuppressive therapies. A high unmet medical need remains for patients with refractory or chronic relapsing wAIHA. Neonatal Fc receptor (FcRn) blockers are novel biologic therapies designed to provide a rapid, sustained decrease in circulating concentrations of IgG antibodies, including autoantibodies, and have been investigated in hematologic conditions like immune thrombocytopenia and hemolytic anemia of the fetus and newborn and other autoimmune conditions, such as generalized myasthenia gravis. FcRn blockade is currently under evaluation in patients with wAIHA to determine its potential as a safe, effective treatment option.
Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea.
Autoimmune hemolytic anemia (AIHA) is rare and characterized by hemolytic anemia with a positive direct antiglobulin test result after the exclusion of other causes. While adults often relapse within 1 year of first-line steroid therapy, children generally respond well. However, current treatment approaches lack substantial evidence and are primarily expert opinion-based. This study aimed to contribute our single-center experience to pediatric AIHA treatment guidelines. Between January 2012 and June 2024, 475 children were diagnosed with anemia; of them, 18 had immune hemolytic anemia, including 6 with neonatal alloimmune hemolytic anemia, 2 who were treated at other centers, and 2 with transient bone marrow suppression due to a viral infection. Thus, this study retrospectively analyzed the treatment responses of 8 patients with AIHA. The median age at diagnosis was 5.2 years (range, 2.3-11.8 years); 62.5% (5 of 8) were male. Median hemoglobin (Hb) at diagnosis was 6.3 g/dL (range, 3.4-9.5 g/dL), median reticulocyte index was 6.53% (range, 1.64%-22.07%), median total bilirubin was 2.75 mg/dL (range, 0.98-7.23 mg/dL), and median lactate dehydrogenase was 1,662 U/L (range, 790-2,921 U/L). All haptoglobin levels were <10 mg/dL. Treatments included steroids (8 of 8), red blood cell transfusions (5 of 8), and intravenous immunoglobulins (2 of 8). Half of the steroid-treated patients received intravenous methylprednisolone for 1-5 days, while half received oral prednisolone (median, 1.78 [range, 0.79-3.39] mg/kg/day). The median time to age-adjusted normal Hb levels was 16.5 days (range, 9.0-22.0 days). Steroids were administered for a median 37.5 days (range, 14.0-119.0 days). Excluding one patient later diagnosed with systemic lupus erythematosus, no relapses occurred during the 3- to 19-month follow-up period. Patients with pediatric AIHA showed relapsefree rapid hematological improvement and sustained steroid responses within 2 months, suggesting that systematic steroid treatment is feasible and highlighting the need for multicenter trials to establish standardized guidelines.
Newborn Screening for Hurler Syndrome Facilitates Early Transplant and Good Outcomes.
Hematopoietic cell transplantation (HCT) is the standard of care treatment for children with Hurler syndrome (HS). This study describes the impact of newborn screening (NBS) on HCT outcomes for these patients. Retrospective study of HS patients diagnosed through NBS and referred to Duke from 2017 to 2023. Patients received a myeloablative busulfan-based regimen and unrelated umbilical cord blood HCT, with cyclosporine and mycophenolate for graft-versus-host-disease prophylaxis. Patients (N =9) were transplanted at a median age of 5.2 months and median weight of 7.8 kg. Median reinfused total nucleated cell was 14.8 × 107/kg. The median times to neutrophil and platelet engraftment were 17 and 48 days, respectively. No primary graft failures or rejections were observed. Post-HCT complications included sinusoidal obstructive syndrome, microangiopathy and autoimmune hemolytic anemia. At median follow-up of 29.1 months (range 4.1-72.2), 8 of 9 patients were alive with normal alpha-L-iduronidase (IDUA) levels, Lansky scores of 90-100%, and developing milestones. One patient died due to autoimmune hemolytic anemia on day +139 (with normal IDUA level and >98% donor chimerism at day +100). Early umbilical cord blood transplant during infancy of HS patients diagnosed through NBS is safe, feasible, and corrects IDUA enzyme deficiency. Follow-up studies will ascertain the long-term benefits of this approach.
Management of autoimmune hemolytic anemia.
Autoimmune hemolytic anemia (AIHA) is caused by premature erythrocyte destruction mediated by autoantibodies (auto-Ab) with or without complement activation. The most frequent form (60%-70% of cases) is warm AIHA (wAIHA), driven by immunoglobulin G auto-Ab that react at body temperature. Cold agglutinin disease (CAD, 20%-25%) is the second most common form and is caused by immunoglobulin M auto-Ab that usually react at temperatures <20°C and strongly activate complement. Rarer forms (5%-10%) include mixed AIHAs (wAIHA plus CAD), and paroxysmal cold hemoglobinuria. Here, we present the management of wAIHA, as CAD is discussed separately. Approximately 50% of wAIHA are primary, whereas the remainder are secondary to various conditions (infections, lymphoproliferative disorders, systemic or organ-specific autoimmune diseases, congenital immunodeficiencies, hematopoietic stem-cell transplantation, and several drugs, including immune checkpoint inhibitors). The disease is highly heterogeneous, ranging from fully compensated to life-threatening, and frequently has a relapsing course. Standard first-line therapy includes steroids with or without intravenous immunoglobulin, transfusions when anemia is clinically significant, prophylactic anticoagulation for severe hemolysis, and recombinant erythropoietin when reticulocytopenia/inadequate bone marrow compensation is present. For severe cases, high-dose steroids and plasma-exchange may be considered. Rituximab is now the preferred second-line option for relapsed/refractory patients, comparing favorably with the traditional splenectomy. The latter is increasingly reserved for later lines together with classic immunosuppressants. Several novel treatments are in development for refractory wAIHA, encompassing drugs targeting B-cells (parsaclisib, ibrutinib, rilzabrutinib, zanubrutinib, obexelimab, ianalumab, povetacicept), plasma cells (bortezomib, daratumumab), spleen tyrosine kinase (fostamatinib, sovleplenib), and the neonatal Fc receptor (nipocalimab).
Publicações recentes
Management of autoimmune hemolytic anemia.
[Future directions in the management of warm autoimmune hemolytic anemia].
Beneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.
Targeting the neonatal Fc receptor (FcRn) in hematologic conditions with a focus on warm autoimmune hemolytic anemia.
Splenectomy following rituximab and cyclophosphamide for severe autoimmune hemolytic anemia in pregnancy.
📚 EuropePMC1 artigos no totalmostrando 78
Autoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
Blood reviewsManagement of autoimmune hemolytic anemia.
Hematology. American Society of Hematology. Education Program20 years experience of RhD alloimmunization at a state referral center in Rio de Janeiro: a study of 481 cases.
Revista brasileira de ginecologia e obstetricia : revista da Federacao Brasileira das Sociedades de Ginecologia e Obstetricia[Future directions in the management of warm autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyFcRn Blockade as a Targeted Therapeutic Strategy in Antibody-Mediated Autoimmune Diseases: A Focus on Warm Autoimmune Hemolytic Anemia.
Antibodies (Basel, Switzerland)Beneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.
Frontiers in immunologyTargeting the neonatal Fc receptor (FcRn) in hematologic conditions with a focus on warm autoimmune hemolytic anemia.
Blood reviewsThe Clinical Significance of Warm Autoantibodies during Pregnancy.
Transfusion medicine (Oxford, England)Splenectomy following rituximab and cyclophosphamide for severe autoimmune hemolytic anemia in pregnancy.
Hematology (Amsterdam, Netherlands)Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea.
Clinical and experimental pediatricsTreatment of autoimmune hemolytic anemia: novel and investigational approaches.
Minerva medicaNeonatal lupus erythematosus: 24 years of experience from a tertiary centre at Chandigarh, North India.
LupusNewborn Screening for Hurler Syndrome Facilitates Early Transplant and Good Outcomes.
Pediatric neurologyAutoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und ImmunhamatologieCurrent state and potential applications of neonatal Fc receptor (FcRn) inhibitors in hematologic conditions.
American journal of hematologyImmunologic hemolytic anemia associated with neonatal alloimmune thrombocytopenia caused by HLA antibody in a male neonate.
Pediatric blood & cancerTreatment with plasma exchange of a pregnant woman with anti-PP1Pk alloimmunization: A case report.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisSuccessful management of severe Kell alloimmunization in pregnancy with intravenous immune globulin.
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Expert opinion on pharmacotherapyAntibody based therapeutics for autoimmune hemolytic anemia.
Expert opinion on biological therapy[Association of giant cell hepatitis and autoimmune hemolytic anemia in infancy].
Orvosi hetilapWhere do they go? The clinical conundrum of warm autoantibodies and their inability to cause haemolytic disease of the foetus and newborn.
British journal of haematologyAutoimmune hemolytic anemia, a rare disease in newborns: a case report.
Annals of medicine and surgery (2012)The First-Reported Case of Drug-Induced Hemolytic Anemia by Piperacillin-Tazobactam in a Premature Neonate: A Case Report and Literature Review.
CureusAutoimmune hemolytic anemia during pregnancy and puerperium: an international multicenter experience.
BloodNot as "D"eadly as once thought - the risk of D-alloimmunization and hemolytic disease of the fetus and newborn following RhD-positive transfusion in trauma.
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Pediatric blood & cancerNew Therapies for the Treatment of Warm Autoimmune Hemolytic Anemia.
Transfusion medicine reviewsA case of severe hemolytic disease of newborn due to alloimmunization in primigravida.
Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguineUnderlying Inborn Errors of Immunity in Patients With Evans Syndrome and Multilineage Cytopenias: A Single-Centre Analysis.
Frontiers in immunologyFetal and Neonatal Reticulocyte Count Response to Intrauterine Transfusion for the Treatment of Red Blood Cell Alloimmunization.
Journal of pediatric hematology/oncologyUpdates in the Management of Warm Autoimmune Hemolytic Anemia.
Hematology/oncology clinics of North AmericaFc receptors gone wrong: A comprehensive review of their roles in autoimmune and inflammatory diseases.
Autoimmunity reviews[Transfusion Strategy for Rare Anti-LW Antibody Autoimmune Hemolytic Anemia Patients with Neonatal Hemolytic Disease].
Zhongguo shi yan xue ye xue za zhiOutcome predictors for maternal red blood cell alloimmunisation with anti-K and anti-D managed with intrauterine blood transfusion.
British journal of haematologySevere neonatal autoimmune thrombocytopenia secondary to maternal Evans syndrome.
BMJ case reportsTransient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report.
Journal of medical case reportsAutoimmune Hemolytic Anaemias in Pregnancy: Experience in a Tertiary Care Hospital in South India.
Journal of obstetrics and gynaecology of IndiaHemolytic Disease of Newborn: Beyond Rh-D and ABO Incompatibility.
Indian pediatrics[Severe foetal anaemia caused by undetected alloimmunisation in a RhD-positive pregnant woman].
Ugeskrift for laeger[Diagnosis of congenital hemolytic anemia by comprehensive gene analysis: significance and limitations].
[Rinsho ketsueki] The Japanese journal of clinical hematologyIs it necessary to add the eluate testing to the direct antiglobulin test to improve the detection of maternal erythrocyte alloantibodies?
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisCytomegalovirus-Associated Hemolytic Anemia in an Infant Born to a Mother with Lupus.
NeonatologyLiving donor liver transplantation for small infants aged less than 6 months: The experience of a single institute.
Journal of pediatric surgeryGiant cell hepatitis associated with autoimmune hemolytic anemia: an update.
Translational gastroenterology and hepatology[An updated advance of autoimmune hepatitis in children].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologySterol 27-Hydroxylase Deficiency as a Cause of Neonatal Cholestasis: Report of 2 Cases and Review of the Literature.
Frontiers in pediatricsA novel zeta-associated protein 70 homozygous mutation causing combined immunodeficiency presenting as neonatal autoimmune hemolytic anemia.
Immunologic researchExpanded utilization of rituximab in paediatric cardiac transplant patients.
Journal of clinical pharmacy and therapeuticsNew Insights in Autoimmune Hemolytic Anemia: From Pathogenesis to Therapy Stage 1.
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The Journal of allergy and clinical immunologyMedical therapy to attenuate fetal anaemia in severe maternal red cell alloimmunisation.
British journal of haematologySirolimus is effective for primary relapsed/refractory autoimmune cytopenia: a multicenter study.
Experimental hematologyRh Alloimmunisation: Current Updates in Antenatal and Postnatal Management.
Indian journal of pediatricsPotential role of Howell-Jolly bodies in identifying functional hyposplenism: a prospective single-institute study.
International journal of hematologyFcRn augments induction of tissue factor activity by IgG-containing immune complexes.
BloodAutoimmune hemolytic anemia in pregnancy: a challenge for maternal and fetal follow-up.
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TransfusionCold reacting anti-M causing delayed hemolytic disease of the newborn.
TransfusionPerinatal outcomes of intrauterine transfusion for foetal anaemia due to red blood cell alloimmunisation.
Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and GynaecologyImmunopathological manifestations in Kabuki syndrome: a registry study of 177 individuals.
Genetics in medicine : official journal of the American College of Medical GeneticsBlood Donation During Pregnancy Due to Anti-Ku Hemolytic Disease of the Fetus and Newborn.
Laboratory medicineB-cell depletion induces prolonged remission in patients with giant cell hepatitis and autoimmune hemolytic anemia.
Clinics and research in hepatology and gastroenterologyNovel compound heterozygous mutations in the SPTA1 gene, causing hereditary spherocytosis in a neonate with Coombs‑negative hemolytic jaundice.
Molecular medicine reportsA rare case of Coombs-negative autoimmune haemolytic anaemia in pregnancy.
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Oncology reportsAnti-Vel alloimmunization and severe hemolytic disease of the fetus and newborn.
ImmunohematologyRisk Factors, Treatment, and Immune Dysregulation in Autoimmune Cytopenia after Allogeneic Hematopoietic Stem Cell Transplantation in Pediatric Patients.
Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow TransplantationRASGRP1 mutation in autoimmune lymphoproliferative syndrome-like disease.
The Journal of allergy and clinical immunologyNext-generation sequencing unravels homozygous mutation in glucose-6-phosphate isomerase, GPIc.1040G>A (p.Arg347His) causing hemolysis in an Indian infant.
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LupusBenefits of Delayed Cord Clamping in Red Blood Cell Alloimmunization.
PediatricsMean reticulocyte volume enhances the utility of red cell mean sphered cell volume in differentiating peripheral blood spherocytes of hereditary spherocytosis from other causes.
Indian journal of pathology & microbiologyThe clinical features of autoimmunity in 53 patients with Wiskott-Aldrich syndrome in China: a single-center study.
European journal of pediatricsImmune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome associated with neonatal epidermolysis bullosa acquisita.
Pediatric dermatologyCharacterization and review of MTHFD1 deficiency: four new patients, cellular delineation and response to folic and folinic acid treatment.
Journal of inherited metabolic diseaseAssociações
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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.
- Autoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
- Targeting the neonatal Fc receptor (FcRn) in hematologic conditions with a focus on warm autoimmune hemolytic anemia.
- Treatment and clinical outcomes of pediatric autoimmune hemolytic anemia: real-world single-center data from Korea.
- Newborn Screening for Hurler Syndrome Facilitates Early Transplant and Good Outcomes.
- Management of autoimmune hemolytic anemia.
- [Future directions in the management of warm autoimmune hemolytic anemia].
- Beneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.
- Splenectomy following rituximab and cyclophosphamide for severe autoimmune hemolytic anemia in pregnancy.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:398109(Orphanet)
- MONDO:0018358(MONDO)
- GARD:21645(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787991(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
