Uma forma adquirida de hemofilia A, que resulta em sangramento espontâneo em indivíduos sem histórico de distúrbios hemorrágicos. Acredita-se que seja causada pela inibição espontânea do fator de coagulação VIII por autoanticorpos, e está habitualmente associada a outras condições autoimunes.
Introdução
O que você precisa saber de cara
Visão geral
A Hemofilia A adquirida (código MONDO: MONDO:0035735, CID-10: D68.4) é uma condição adquirida caracterizada por sangramento espontâneo em indivíduos sem histórico prévio de distúrbios hemorrágicos. Acredita-se que o quadro decorra da inibição espontânea do fator VIII de coagulação por autoanticorpos, encontrando-se habitualmente relacionada a outras condições autoimunes.[1]
A prevalência desta condição é desconhecida. Quanto à faixa etária de início, a manifestação pode ocorrer na infância, adolescência, idade adulta ou em idosos.[1]
Causas genéticas
Tratamento e manejo
Tratamentos citados na literatura
As seguintes substâncias foram identificadas em associações mineradas na literatura científica e não configuram recomendações de tratamento: Rituximab (97 publicações), Cyclophosphamide (94 publicações), Prednisolone (51 publicações), Prednisone (48 publicações), Steroids (42 publicações), emicizumab (37 publicações), Methylprednisolone (16 publicações), Cyclosporine (11 publicações), Azathioprine (7 publicações) e Bortezomib (5 publicações).[3]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
A Hemofilia A adquirida é uma condição rara em que o organismo cria autoanticorpos que inibem o fator VIII da coagulação do próprio corpo. Ela se manifesta por sangramentos espontâneos e inesperados em pessoas que nunca tiveram problemas prévios de coagulação. Ao contrário da hemofilia comum, ela não é herdada geneticamente e costuma estar associada a outras doenças autoimunes. No SUS, a cobertura assistencial é classificada como parcial e o documento ministerial existente para hemofilia não cobre formalmente esta forma adquirida.
Uma forma adquirida de hemofilia A, que resulta em sangramento espontâneo em indivíduos sem histórico de distúrbios hemorrágicos. Acredita-se que seja causada pela inibição espontânea do fator de coagulação VIII por autoanticorpos, e está habitualmente associada a outras condições autoimunes.
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)
🇧🇷 Atendimento SUS — Hemofilia A adquirida
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Com ensaio aberto em 3 países. O ponto verde marca onde há vaga agora.
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Outros ensaios clínicos
14 ensaios clínicos encontrados, 5 ativos.
Publicações mais relevantes
Delayed diagnosis of acquired hemophilia a complicating hepatocellular carcinoma: a rare case of hepatectomy following atezolizumab and bevacizumab therapy.
Hemorrhagic Shock and Massive Abdominal Wall Hematoma After Cesarean Section in a Patient with Peripartum Acquired Hemophilia A:A Case Report.
Acquired Hemophilia A (AHA), a rare bleeding disorder caused by autoimmune suppression of coagulation factor VIII(FVIII), has a low incidence in perinatal women but carries significant risks of severe hemorrhage. The lack of consensus guidelines and limited clinical data from developing countries underscore the importance of this case report. Consequently, this report informs clinical practice and expands the evidence base for the treatment of AHA. This case describes an extensive abdominal wall hematoma and hemorrhagic shock following a cesarean section in a patient. The condition was successfully managed through comprehensive interventions, including shock resuscitation, endovascular embolization, pharmacological hemostatic therapy, removal of circulating immune complexes, and immunosuppressive therapy. AHA usually causes acute bleeding, and early identification and removal of acquired inhibitors are the main treatment methods. In such cases, endovascular embolization is an effective procedure to control bleeding.
Clinical Experience with Emicizumab and Rituximab as First-Line Treatment in a Case Series of Acquired Hemophilia A.
Background: Acquired hemophilia A (AHA) is a bleeding disorder caused by autoantibodies against coagulation factor VIII. Treatment includes controlling bleeding and eliminating the inhibitor. Emicizumab has been increasingly used to prevent bleeding in patients with AHA. Rituximab is used as a first-line immunosuppressive therapy (IST) for AHA, either in combination with corticosteroids in high-risk patients or as monotherapy in low-risk patients who cannot tolerate corticosteroids. However, evidence regarding concomitant emicizumab and rituximab as first-line treatment for AHA is limited. Case presentations: We present five cases of AHA diagnosed at a single institution. The first three high-risk AHA cases in the era before emicizumab resulted in poor outcomes due to bleeding (Cases 1 and 3) or infection (Case 2). The recent cases (Cases 4 and 5) were successfully treated with emicizumab and rituximab-containing IST without severe bleeding and infections. Since emicizumab effectively relieved pain in these patients, rehabilitation could be initiated promptly, resulting in earlier hospital discharge. Complete remission was achieved on Day 42 in Case 4 and on Day 22 in Case 5, respectively, and emicizumab was subsequently discontinued in both cases. Conclusions: Our case series suggests that early initiation of emicizumab for patients with AHA is effective in preventing severe bleeding and subsequent immobility, and it can be combined with rituximab-containing IST to achieve remission, potentially with fewer adverse effects than standard IST. Further studies are warranted to establish the optimal treatment protocol involving emicizumab and IST for AHA.
Case Report: Differential diagnosis and clinical management of isolated prolonged activated partial thromboplastin time.
An unexpected, isolated prolongation of activated partial thromboplastin time (APTT) frequently poses diagnostic challenges in coagulation laboratories. This article presents two patients exhibiting isolated prolonged APTT, one with significant bleeding manifestations and another without bleeding symptoms. The first patient was diagnosed with acquired hemophilia A (AHA), whereas antiphospholipid antibodies (aPLs) were suspected to be responsible for the isolated prolonged APTT in the second patient. Accurate identification of the underlying cause of isolated prolonged APTT is crucial for proper diagnosis and subsequent therapeutic management. Furthermore, we summarize potential causes of isolated prolonged APTT and propose a simplified diagnostic algorithm to distinguish between lupus anticoagulants (LA) (more common) and coagulation factor deficiencies (less common). This report aims to provide insights into the clinical management of similar cases in the future.
[Acquired hemophilia A secondary to marginal zone lymphoma].
Acquired hemophilia A (AHA) is a rare but potentially life-threatening disorder characterized by the development of inhibitors against factor VIII. In 30-50 % of cases, AHA is associated with an underlying condition. We present a case of acquired hemophilia A secondary to marginal zone lymphoma. The diagnosis of AHA should raise suspicion for an underlying neoplastic process, prompting a thorough diagnostic workup. Although etiology remains unidentified in approximately half of AHA cases, it is crucial to maintain a high index of suspicion for malignancy, as neoplastic diseases may emerge even after treatment of AHA. The management of acquired hemophilia A consists of three main objectives : control of active bleeding, eradication of the inhibitor, and treatment of the underlying disease. Ce cas clinique rapporte un patient atteint d’hémophilie A acquise secondaire à un lymphome de la zone marginale. L’hémophilie A acquise est une coagulopathie rare et potentiellement mortelle, liée au développement d’inhibiteurs acquis du facteur VIII. Cette pathologie est, dans 30 à 50 % des cas, secondaire à une affection sous-jacente. Le diagnostic d’hémophilie A acquise doit faire suspecter une pathologie potentiellement néoplasique et impose la réalisation d’un bilan complémentaire approfondi. Les explorations étiologiques restent négatives dans près de la moitié des cas, mais le développement ultérieur d’une pathologie néoplasique reste possible même après le traitement de l’hémophilie A acquise. Le traitement repose sur trois objectifs principaux : le contrôle de l’éventuel saignement actif, l’élimination de l’inhibiteur et le traitement spécifique de la pathologie sous-jacente.
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📚 EuropePMC787 artigos no totalmostrando 199
Hemorrhagic Shock and Massive Abdominal Wall Hematoma After Cesarean Section in a Patient with Peripartum Acquired Hemophilia A:A Case Report.
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The American journal of case reportsAcquired Hemophilia A Diagnosed Based on Gross Hematuria: A Case Report and Literature Review.
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Emergencias : revista de la Sociedad Espanola de Medicina de EmergenciasEmicizumab as a Promising Form of Therapy for Type A Hemophilia - A Review of Current Knowledge from Clinical Trials.
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Expert review of hematologyConcurrent congenital hemophilia B and acquired hemophilia A: a unique case report.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisNavigating challenges in diagnosing acquired hemophilia A: A case report from Syria.
Clinical case reportsAdvanced case of Waldenström macroglobulinemia complicated by acquired hemophilia A in a patient with neurofibromatosis type 1.
Polish archives of internal medicineEmicizumab for acquired hemophilia A: Report of two cases and dosing strategies.
EJHaemCyclophosphamide vs rituximab for eradicating inhibitors in acquired hemophilia A: A randomized trial in 108 patients.
Thrombosis researchAcquired Hemophilia: A Rare Complication of Pediatric Idiopathic Multicentric Castleman Disease.
PediatricsAcquired factor VIII deficiency in a nulliparous patient undergoing induction of labor.
Case reports in perinatal medicineAcquired hemophilia A: a single-center study of 165 patients.
Research and practice in thrombosis and haemostasisInnovative Therapies for Acquired Hemophilia A.
Seminars in thrombosis and hemostasisAberrant Right Subclavian Artery Complicated by Acquired Hemophilia A and a Subclavian Artery-Esophageal Fistula after Traumatic Injury.
Journal of emergencies, trauma, and shockHematological Complications in a COVID-19 Patient: A Case Report.
Diseases (Basel, Switzerland)Postpartum-acquired hemophilia A: case report.
Revista peruana de medicina experimental y salud publicaAcquired Hemophilia A After Multiple Transfusions Following Trauma.
CureusDiagnostic Challenges and Management of Acquired Hemophilia A Presenting as Gross Hematuria: A Case Report.
CureusCombined life-threatening internal organ bleeding and postpartum hemorrhage associated with acquired hemophilia A.
Clinical case reportsAcquired hemophilia A treated with rituximab in a 62-year-old female with rheumatoid arthritis: a case-based review.
ReumatismoDiagnosis and laboratory monitoring of acquired hemophilia A.
Hematology. American Society of Hematology. Education ProgramThe role of emicizumab in acquired hemophilia A.
Hematology. American Society of Hematology. Education ProgramImmunotherapy of acquired hemophilia A.
Hematology. American Society of Hematology. Education Program[Profuse Hemorage During Open Heart Surgery in a Patient Late After Liver Transplantation and Remission Stage Acquired Hemophilia].
Kyobu geka. The Japanese journal of thoracic surgeryEmicizumab for the Treatment of Acquired Hemophilia A: Consensus Recommendations from the GTH-AHA Working Group.
HamostaseologieCharacteristics and outcome of a territory-wide cohort study of patients with acquired hemophilia A in Hong Kong.
Thrombosis researchAcquired hemophilia A induced by clopidogrel.
Panminerva medicaAcquired Hemophilia A: A Rare, Acquired Coagulopathy in the Postpartum Setting.
AJP reportsKey Genes of the Immune System and Predisposition to Acquired Hemophilia A: Evidence from a Spanish Cohort of 49 Patients Using Next-Generation Sequencing.
International journal of molecular sciences[Rapid diagnosis of acquired hemophilia A with ClotPro viscoelastometry analyser].
Orvosi hetilapAcquired Hemophilia A: Rare Cause of Upper Airway Hematoma and a Literature Review.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaA bispecific antibody NXT007 exerts a hemostatic activity in hemophilia A monkeys enough to keep a nonhemophilic state.
Journal of thrombosis and haemostasis : JTH[Diagnosis and treatment of autoimmune acquired coagulation factor deficiency].
[Rinsho ketsueki] The Japanese journal of clinical hematologyAcquired Hemophilia A: Bleeding Pattern and Hemostatic Therapeutic Strategies.
Medicina (Kaunas, Lithuania)Single-dose rituximab plus glucocorticoid versus cyclophosphamide plus glucocorticoid in patients with newly diagnosed acquired hemophilia A: A multicenter, open-label, randomized noninferiority trial.
American journal of hematologyLow-Dose Cyclophosphamide Associated With Hyponatremia and Hepatotoxicity.
CureusThe Nijmegen ultra-sensitive Bethesda Assay detects very low-titer factor VIII inhibitors in patients with congenital and acquired hemophilia A.
Thrombosis researchAcquired Hemophilia A after SARS-CoV-2 Infection: A Case Report and an Updated Systematic Review.
BiomedicinesA Rare Case of Acquired Factor VIII Deficiency in an Elderly Male With a History of Rheumatoid Arthritis.
CureusComprehensive comparison of global coagulation assays to differentiate lupus anticoagulant from acquired hemophilia A in patients with prolonged APTT.
International journal of hematologyAn Unusual Case of Bleeding: Acquired Hemophilia A.
CureusAcquired bleeding disorders secondary to immune checkpoint inhibitors: a case report and systematic literature review.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisA 10-year single-institution retrospective review of treatment for acquired hemophilia A.
Haemophilia : the official journal of the World Federation of HemophiliaLevel of Awareness of Acquired Hemophilia A Among Physicians in Türkiye: A Survey Study.
Turkish journal of haematology : official journal of Turkish Society of HaematologyThe diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa.
Journal of primary care & community healthA case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma.
Biomedical reportsThe safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisSystemic lupus erythematosus with acquired hemophilia A: A case report and literature review.
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciencesReal-world data in patients with congenital hemophilia and inhibitors: final data from the FEIBA Global Outcome (FEIBA GO) study.
Therapeutic advances in hematologyAcquired Hemophilia A Masquerading as Bleeding on Anticoagulation: A Case Report Including Key Laboratory Considerations.
CureusSurgery and Prophylaxis with Susoctocog-Alfa in Acquired Hemophilia: Case Series and Literature Review.
Journal of clinical medicineAlemtuzumab induced acquired hemophilia A in multiple sclerosis: a case report.
Annals of hematologyCombining Low-Dose Rituximab and Bortezomib as Immunosuppressive Therapy for Acquired Hemophilia A: 6 Case Series.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionBullous pemphigoid associated with acquired hemophilia A.
Clinics in dermatologyAcquired Hemophilia A and ST-Elevation Myocardial Infarction (STEMI): A Rare Presentation and Management Dilemma.
CureusA Case Report of Idiopathic Acquired Hemophilia Type A.
Cureus[Utility of thromboelastography in the treatment of acquired hemophilia A].
[Rinsho ketsueki] The Japanese journal of clinical hematologyCyclosporine treatment for steroid-resistant nephrosis complicated with acquired hemophilia A: a case report.
Annals of medicine and surgery (2012)Acquired hemophilia A (AHA) due to anti-SARS-CoV-2 vaccination: A systematic review.
EJHaemStructure of coagulation factor VIII bound to a patient-derived anti-C1 domain antibody inhibitor.
BloodAcquired hemophilia A in a Taiwanese patient with bullous pemphigoid.
International journal of rheumatic diseasesAcquired hemophilia A developing in the perioperative period of pancreatoduodenectomy: a report of two cases.
Surgical case reportsSudden-onset gallbladder rupture due to Ceftriaxone-associated pseudolithiasis in a patient with acquired hemophilia A.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyA case of refractory bleeding from duodenal angioectasia with acquired hemophilia A.
Clinical journal of gastroenterologyAcquired Hemophilia A: A Case Report on a Rare Disease Manifesting as Persistent Hematuria.
CureusAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Hemofilia A adquirida.
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Hemofilia A adquirida e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Existe um documento oficial para "Hemofilia A e B". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
A condição é causada pela produção de autoanticorpos que inibem espontaneamente o fator de coagulação VIII. Ela é habitualmente associada a outras doenças autoimunes e não possui herança genética.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
4 fontes citadas no texto · 10 publicações do grafo RarasNet (PubMed) · 4 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Hemofilia A adquirida. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hemofilia-a-adquirida
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hemofilia A adquirida
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
