A anemia hemolítica autoimune quente é a forma mais comum de anemia hemolítica autoimune definida pela presença de autoanticorpos quentes contra glóbulos vermelhos (autoanticorpos ativos em temperaturas entre 37-40°C).
Introdução
O que você precisa saber de cara
A anemia hemolítica autoimune quente é a forma mais comum de anemia hemolítica autoimune definida pela presença de autoanticorpos quentes contra glóbulos vermelhos (autoanticorpos ativos em temperaturas entre 37-40°C).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
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Partes do corpo afetadas
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Características mais comuns
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Publicações mais relevantes
A Complex Case of Anti-e Mimicking Autoantibody Development Leading to Warm Autoimmune Hemolytic Anemia: Case Report.
Newly diagnosed autoimmune hemolytic anemia (AIHA) develops in 1-3 individuals per 100,000 people per year. Warm autoimmune hemolytic anemia (WAIHA) is the most common type of AIHA, accounting for approximately 70-80% of cases. This report outlines complex presentation of DTA-negative, anti-e-mimicking autoantibody mediated WAIHA following a transient viral infection in an adult male patient with no prior transfusions. A 55-year-old male patient presented with several weeks of flu-like symptoms followed by jaundice, dyspnea, and fatigue with lab work up notable initially for an anti-C antibody mediated WAIHA but later determined to be an anti-e mimicking pan-agglutinating autoantibody. This report highlights a complex case of a patient with an autoantibody mimicking anti-e associated WAIHA and the challenge of identifying suitable transfusion options.
When Warm Meets Cold: Mixed-Type Autoimmune Hemolytic Anemia Revealing an Underlying Autoimmune Disorder.
Autoimmune hemolytic anemia (AIHA) is a rare immune-mediated disorder caused by autoantibodies directed against red blood cell antigens. Mixed-type AIHA, characterized by the coexistence of warm-reactive IgG and cold-reactive complement-fixing antibodies, is uncommon and often diagnostically challenging due to overlapping serologic features and transfusion incompatibility. We report the case of a 40-year-old woman who presented with an acute onset of breathlessness, palpitations, and severe fatigability. She was found to have severe anemia (hemoglobin 2.7 g/dL), reticulocytosis, indirect hyperbilirubinemia, and red cell agglutination on peripheral smear. Both direct and indirect Coombs tests were strongly positive, while antibody screening demonstrated panreactivity with positive autocontrol. The monospecific direct antiglobulin test confirmed positivity for IgG and C3d, establishing the diagnosis of mixed-type AIHA. In view of hemodynamic instability, transfusion with the least-incompatible packed red cells was performed under warming precautions, alongside high-dose intravenous corticosteroids. The patient improved symptomatically, with hemoglobin rising to 8.8 g/dL. Autoimmune workup revealed high-titer antinuclear antibody and anti-SSA positivity, suggesting an evolving systemic autoimmune disorder. Mixed-type AIHA accounts for less than 10% of cases but is associated with significant morbidity due to combined extravascular and complement-mediated hemolysis, often necessitating cautious transfusion of least-incompatible blood in life-threatening anemia and escalation to rituximab or other immunosuppressive agents. This case highlights the diagnostic complexity, therapeutic challenges, and need for long-term autoimmune surveillance in patients with mixed-type AIHA.
A Rare Autoimmune Hemolytic Overlap: Coombs-Positive Pernicious Anemia.
Pernicious anemia is a type of autoimmune disease caused by the presence of anti-intrinsic factor and antiparietal cell antibodies, leading to vitamin B12 deficiency and ineffective erythropoiesis. Autoimmune hemolytic anemia is a variant of anemia in which the direct Coombs test is positive. It is uncommon to find a mixed anemia that has both a pernicious anemia component and an autoimmune anemia component with a positive direct Coombs test, as this association is rarely described. Two cases in which this association was found are presented. The first case involved a 65-year-old woman presenting with profound anemia, macrocytosis, reticulocytosis, elevated lactate dehydrogenase, low haptoglobin, and a positive direct Coombs test. She had anti-intrinsic factor antibodies and responded to vitamin B12 supplementation and corticosteroids. The second case involved an 83-year-old woman with severe anemia, vitamin B12 deficiency, positive antiparietal and anti-intrinsic factor antibodies, and positive direct Coombs test, who similarly improved with cobalamin and immunosuppressive therapy. These cases highlight the rare overlap of pernicious anemia and autoimmune hemolytic anemia, emphasizing the importance of recognizing concurrent autoimmune hemolysis in patients with severe vitamin B12 deficiency to guide prompt and appropriate therapy.
Transfusion-induced hemolysis in warm autoimmune hemolytic anemia with autoantibodies that have anti-e specificity: a case report and systematic review of the literature.
Warm autoimmune hemolytic anemia (wAIHA) is characterized by autoantibodies against proteins that comprise the Rh antigen system along with hemolysis. The presence of specific autoantibodies, especially autoantibodies with anti-e specificity, is rare. The pathophysiology, management, and outcomes in this condition, including with transfusion of e-antigen positive packed red blood cells (pRBCs), remain poorly understood. A 63-year-old woman presented with fatigue, jaundice, tea-colored urine, and dyspnea. She was diagnosed with wAIHA with anti-e specificity and developed worsening hemolysis after receiving a unit of e-antigen positive pRBCs with a drop in hemoglobin from 85 to 71 g/L. She achieved a complete response with a tapering course of prednisone and folate. We conducted a systematic review of the literature by searching MEDLINE, EMBASE, and Cochrane for articles on management of wAIHA with anti-e specificity published between 1946 and 2025. We identified eighteen cases of wAIHA mediated through autoantibodies with anti-e specificity, only one of which demonstrated hemolysis after transfusion with e-antigen positive pRBCs. Treatment with corticosteroids and rituximab was effective in many of these cases. This is the one of the few cases to demonstrate increased hemolysis after transfusion with e-antigen positive pRBCs in a patient with wAIHA and autoantibodies with anti-e specificity. In these patients, transfusion of e-antigen positive pRBCs should be avoided and if transfusion is necessary, it will be important to closely monitor for worsening hemolysis. Additionally, our case and those reported in the literature highlight the effectiveness of standard corticosteroid treatment for this rare type of wAIHA.
Disseminated intravascular coagulation secondary to postpartum hemorrhage complicated with autoimmune hemolytic anemia: A case report.
This study reported a case of disseminated intravascular coagulation secondary to postpartum hemorrhage complicated by autoimmune hemolytic anemia. A pregnant woman in her early 40s was admitted to The Third Central Hospital of Tianjin with regular contractions in July 2022. The direct antiglobulin test was positive for IgG and C3d. Following the delivery of a baby girl, she developed severe postpartum hemorrhage, and the obstetric disseminated intravascular coagulation score exceeded 8 points. She was diagnosed with disseminated intravascular coagulation secondary to postpartum hemorrhage and warm-antibody type autoimmune hemolytic anemia (IgG + C3). The patient underwent methylprednisone therapy, emergency selective bilateral uterine artery embolization, and transfusion with suspended and washed red blood cells. Subsequently, routine coagulation parameters normalized, and the urine color gradually cleared. Therefore, glucocorticoids, uterine artery embolization, and blood transfusion may be effective for managing disseminated intravascular coagulation secondary to postpartum hemorrhage complicated by autoimmune hemolytic anemia.
Publicações recentes
Delayed-Onset Autoimmune Hemolytic Anemia in Advanced HIV With Cerebral Toxoplasmosis.
[Rapid response to linperlisib in relapsed/refractory autoimmune hemolytic anemia: a case report].
[Treatment practice and outcomes in 125 patients with autoimmune hemolytic anemia: a single-center retrospective analysis].
Case Report: Refractory cold agglutinin disease with hypersplenism: efficacy of splenectomy in a patient treated with sutimlimab.
Zieve's Syndrome in a Patient With Alcohol Use Disorder and Alcohol-Associated Cirrhosis: A Case Report and Review of the Literature.
📚 EuropePMC2 artigos no totalmostrando 91
A Complex Case of Anti-e Mimicking Autoantibody Development Leading to Warm Autoimmune Hemolytic Anemia: Case Report.
Acta haematologicaWhen Warm Meets Cold: Mixed-Type Autoimmune Hemolytic Anemia Revealing an Underlying Autoimmune Disorder.
CureusA Rare Autoimmune Hemolytic Overlap: Coombs-Positive Pernicious Anemia.
CureusTransfusion-induced hemolysis in warm autoimmune hemolytic anemia with autoantibodies that have anti-e specificity: a case report and systematic review of the literature.
Therapeutic advances in hematologyDisseminated intravascular coagulation secondary to postpartum hemorrhage complicated with autoimmune hemolytic anemia: A case report.
The Journal of international medical researchA Case Report of a 14-year-old Boy with Mixed-type Autoimmune Hemolytic Anemia.
Annals of African medicineMixed-type autoimmune hemolytic anaemia complicated by acute cerebral infarction: a case report.
Frontiers in medicineBone marrow microenvironment in autoimmune hemolytic anemia: from trephine biopsy to single cell RNA sequencing.
Signal transduction and targeted therapySuccessful Dostarlimab Rechallenge Following Pembrolizumab-Induced Autoimmune Hemolytic Anemia: A Case Report.
Clinical case reportsAutoimmune Hemolytic Anemia as an Initial Manifestation of Splenic Marginal Zone Lymphoma.
CureusThe Clinical Significance of Warm Autoantibodies during Pregnancy.
Transfusion medicine (Oxford, England)Mixed Autoimmune Hemolytic Anemia: A Systematic Review of Epidemiology, Clinical Characteristics, Therapies, and Outcomes.
American journal of hematologyPrevalence of systemic lupus erythematosus in autoimmune hemolytic anemia patients based on coombs test results.
European journal of medical researchThe treatment of autoimmune hemolytic anemia with complement inhibitor iptacopan: a case report.
Frontiers in medicineClinical Characteristics of Patients With Idiopathic Multicentric Castleman Disease Complicated by Autoimmune Hemolytic Anemia.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeInsight Into Aetiology and Severity of Hemolysis Associated with Immunoproteins on Red Cell Surface in Direct Antiglobulin Test Positive Auto-immune Hemolytic Anemia.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionVery low doses of rituximab in autoimmune hemolytic anemia-an open-label, phase II pilot trial.
Frontiers in medicineAutoimmune haemolytic anaemias.
Nature reviews. Disease primersParsaclisib for the treatment of primary autoimmune hemolytic anemia: Results from a phase 2, open-label study.
American journal of hematologyMixed autoimmune hemolytic anemia as the initial presentation of systemic lupus erythematosus: A case report and review.
EJHaem[Current recommendations for the diagnosis and treatment of autoimmune hemolytic anemia based on etiology and pathology].
[Rinsho ketsueki] The Japanese journal of clinical hematologySutimlimab for Cold Agglutinin Disease.
Journal of the advanced practitioner in oncologyCold Agglutinin Disease: A Rare Paraneoplastic Manifestation of a Thyroid Malignancy.
Cureus[The treatment strategies of autoimmune hemolytic anemia].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiDiagnosis and management of Evans syndrome in adults: first consensus recommendations.
The Lancet. Haematology[Cold agglutinin disease: pathology, diagnosis, and treatment].
[Rinsho ketsueki] The Japanese journal of clinical hematologyRole of direct antiglobulin test in anemia under evaluation: Its prevalence, laboratory workup, and significance in further patient management-A study from eastern India.
Indian journal of pathology & microbiologyHemolytic Anemia as a Provoking Factor for Recurrent Venous Thromboembolism: A Case Report.
CureusClinical profile and serological correlation with haemolysis in DAT-positive autoimmune haemolytic anaemia patients in Bangladesh.
PathologySuspect the Unexpected: A Rare Association of Autoimmune Hemolytic Anemia and Hemophagocytic Lymphohistiocytosis with Visceral Leishmaniasis: A Case Report and Review of Literature.
EJIFCCSuccessful Treatment of Warm Autoimmune Hemolytic Anemia with a Positive Donath-Landsteiner Test Using Rituximab.
Internal medicine (Tokyo, Japan)Antibody based therapeutics for autoimmune hemolytic anemia.
Expert opinion on biological therapyTotal arch replacement for an aortic arch aneurysm with cold agglutinin disease after rituximab and plasmapheresis.
Journal of cardiothoracic surgerySystemic Lupus Erythematosus and Antiphospholipid Syndrome Accompanied by Mixed-Type Autoimmune Hemolytic Anemia.
Case reports in rheumatologyGenome-wide DNA methylation profiles analysis in primary warm autoimmune hemolytic anemia patients.
Hematology (Amsterdam, Netherlands)Molecular pharmacology in complement-mediated hemolytic disorders.
European journal of haematologyMixed Warm and Cold Autoimmune Hemolytic Anemia With Concomitant Immune Thrombocytopenia Following Recent SARS-CoV-2 Infection and Ongoing Rhinovirus Infection.
CureusExacerbation of autoimmune hemolytic anemia associated with pure red cell aplasia after COVID-19: A case report.
Journal of infection and chemotherapy : official journal of the Japan Society of ChemotherapyAutoimmune Hemolytic Anemia in Children: Clinical Profile and Outcome.
Indian journal of pediatricsAutoimmune haemolytic anaemia.
Medicina clinicaType O blood, the MCHC, and the reticulocyte count impact the early recurrence of primary warm-antibody autoimmune hemolytic anemia in children: A retrospective cohort analysis.
Frontiers in pediatricsNew Therapies for the Treatment of Warm Autoimmune Hemolytic Anemia.
Transfusion medicine reviewsPractical therapy for primary autoimmune hemolytic anemia in adults.
Clinical and experimental medicine[Thrombotic risk in autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyAutoimmune hemolytic anemia: causes and consequences.
Expert review of clinical immunology[Development of severe aplastic anemia during steroid therapy for autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologySurvival in autoimmune hemolytic anemia remains poor, results from a nationwide cohort with 37 years of follow-up.
European journal of haematologyCOVID-19 and Immune-Mediated RBC Destruction.
American journal of clinical pathologyBlood group discrepancy in mixed-type autoimmune hemolytic anemia in a pediatric patient.
Asian journal of transfusion scienceComplement Mediated Hemolytic Anemias in the COVID-19 Era: Case Series and Review of the Literature.
Frontiers in immunologyCOVID-19-associated Evans syndrome: A case report and review of the literature.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisAn Uncharacteristic Presentation of Evans Syndrome Following Treatment With Dupilumab.
CureusCytomegalovirus-Associated Hemolytic Anemia in an Infant Born to a Mother with Lupus.
NeonatologyA fluorometric erythrophagocytosis assay using differentiated monocytic THP-1 cells to assess the clinical significance of antibodies to red blood cells.
Vox sanguinisClinical Profile and Severity of Hemolysis in Adult Patients of Primary Autoimmune Hemolytic Anemia and Their Response to Steroid: A Prospective Cohort Study from Single Institution.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionAutoimmune hemolytic anemia: current knowledge and perspectives.
Immunity & ageing : I & AAutoimmune Hemolytic Anemia After Cyanocobalamin Replacement in a Patient With a Previous Diagnosis of Pernicious Anemia: A Case Report.
CureusRapidly Exacerbating Autoimmune Hemolytic Anemia Together With Marked Cytokine Storm Triggered by Pneumonia Infection: A Case Report.
Frontiers in immunology[Evaluation of Efficacy and Safety of Blood Transfusion and Hormone Therapy in 40 Patients with Autoimmune Hemolytic Anemia].
Zhongguo shi yan xue ye xue za zhiDifferential red blood cell age fractionation and Band 3 phosphorylation distinguish two different subtypes of warm autoimmune hemolytic anemia.
TransfusionSmall Paroxysmal Nocturnal Hemoglobinuria Clones in Autoimmune Hemolytic Anemia: Clinical Implications and Different Cytokine Patterns in Positive and Negative Patients.
Frontiers in immunologySuccessful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab.
Pediatric gastroenterology, hepatology & nutritionAutoimmune hemolytic anemia in pregnancy: a challenge for maternal and fetal follow-up.
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal ObstetriciansFatal fulminant hemolysis-associated pulmonary embolism in mixed-type autoimmune hemolytic anemia: A case report.
MedicineDiagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting.
Blood reviewsEfficacy and safety of second-line treatment in Thai patients with primary warm-type autoimmune hemolytic anemia.
Hematology (Amsterdam, Netherlands)Autoimmune haemolytic anaemias: A retrospective study of 93 patients.
Medicina clinica[Serological Characteristics of Patients with Autoimmune Hemoly-tic Anemia and Efficacy and Safety of Incompatible Transfusion of Red Blood Cells].
Zhongguo shi yan xue ye xue za zhiAutoimmune hemolytic anemia.
Hematology. American Society of Hematology. Education ProgramAnti-CD20 Treatment of Autoimmune Hemolytic Anemia Refractory to Corticosteroids and Azathioprine: A Pediatric Case Report and Mini Review.
Case reports in hematologyAdsorption Technique in Pre-Transfusion Testing For Patients with Warm Type Autoimmune Hemolytic Anemia.
The Egyptian journal of immunologyCold Agglutinins in Peripheral Blood with Atypical Cells with an Owl-Eye Appearance in Bone Trephine.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPInflammatory Breast Cancer and Warm Antibody Autoimmune Hemolytic Anemia: A Rare Paraneoplastic Syndrome.
Journal of investigative medicine high impact case reportsPlasmapheresis in a child with cold antibody autoimmune hemolytic anemia: case report.
Turk pediatri arsiviRare presentation of mixed autoimmune hemolytic anemia in children: Report of 2 cases.
Journal of laboratory physiciansEryptosis in autoimmune haemolytic anaemia.
European journal of haematologyAlloimmunization in autoimmune hemolytic anemia patient: The differential adsorption approach.
Asian journal of transfusion scienceWarm antibody autoimmune hemolytic anemia.
Hematology. American Society of Hematology. Education ProgramResolution of Serologic Problems Due to Cold Agglutinins in Chronic Lymphocytic Leukemia.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionFatal warm autoimmune hemolytic anemia in a child due to IgM-type autoantibodies.
Pediatrics international : official journal of the Japan Pediatric SocietyClinical characteristics and long-term outcomes of warm-type autoimmune hemolytic anemia.
Hematology (Amsterdam, Netherlands)[Clinical profile of autoimmune hemolytic anemia with monoclonal gammopathy IgMκ].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiAutoimmune Hemolytic Anemia in Children: Mayo Clinic Experience.
Journal of pediatric hematology/oncologyAutoimmune Hemolytic Anemia and Hodgkin's Disease: An Unusual Pediatric Association.
Case reports in pediatricsRole of Complement in Autoimmune Hemolytic Anemia.
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und ImmunhamatologieTreatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review.
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und ImmunhamatologieIL-33 reflects dynamics of disease activity in patients with autoimmune hemolytic anemia by regulating autoantibody production.
Journal of translational medicinePrecautions surrounding blood transfusion in autoimmune haemolytic anaemias are overestimated.
Blood transfusion = Trasfusione del sangue[Autoimmune haemolytic anaemia: a review and report of four cases].
Orvosi hetilapRed blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy.
BioMed research internationalThe effect of erythropoiesis-stimulating agents in patients with therapy-refractory autoimmune hemolytic anemia.
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und ImmunhamatologieAssociações
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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.
- A Complex Case of Anti-e Mimicking Autoantibody Development Leading to Warm Autoimmune Hemolytic Anemia: Case Report.
- When Warm Meets Cold: Mixed-Type Autoimmune Hemolytic Anemia Revealing an Underlying Autoimmune Disorder.
- A Rare Autoimmune Hemolytic Overlap: Coombs-Positive Pernicious Anemia.
- Transfusion-induced hemolysis in warm autoimmune hemolytic anemia with autoantibodies that have anti-e specificity: a case report and systematic review of the literature.
- Disseminated intravascular coagulation secondary to postpartum hemorrhage complicated with autoimmune hemolytic anemia: A case report.
- Delayed-Onset Autoimmune Hemolytic Anemia in Advanced HIV With Cerebral Toxoplasmosis.
- [Rapid response to linperlisib in relapsed/refractory autoimmune hemolytic anemia: a case report].
- [Treatment practice and outcomes in 125 patients with autoimmune hemolytic anemia: a single-center retrospective analysis].
- Case Report: Refractory cold agglutinin disease with hypersplenism: efficacy of splenectomy in a patient treated with sutimlimab.
- Zieve's Syndrome in a Patient With Alcohol Use Disorder and Alcohol-Associated Cirrhosis: A Case Report and Review of the Literature.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:90033(Orphanet)
- MONDO:0019532(MONDO)
- GARD:7876(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q7969590(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.
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