MCID: ACQ042
MIFTS: 37

Acquired Hemophilia a

Categories: Blood diseases, Genetic diseases, Immune diseases, Muscle diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Acquired Hemophilia a

MalaCards integrated aliases for Acquired Hemophilia a:

Name: Acquired Hemophilia a 20
Acquired Factor Viii Deficiency Disease 29
Acquired Factor Vii Deficiency Disease 70
Acquired Factor Vii Deficiency 20
Factor 8 Deficiency, Acquired 70
Acquired Factor 8 Deficiency 20
Acquired Hemophilia 70

Classifications:



External Ids:

UMLS 70 C0272321 C0272325 C1096116

Summaries for Acquired Hemophilia a

GARD : 20 Acquired hemophilia A is a bleeding disorder that interferes with the body's blood clotting process. Although the condition can affect people of all ages, it generally occurs in older people (the median age of diagnosis is between 60 and 67 years). Signs and symptoms include prolonged bleeding, frequent nosebleeds, bruising throughout the body, solid swellings of congealed blood (hematomas), hematuria, and gastrointestinal or urologic bleeding. Acquired hemophilia A occurs when the body's immune system attacks and disables a certain protein that helps the blood clot (called coagulation factor VIII). About half of the cases are associated with other conditions, such as pregnancy, autoimmune disease, cancer, skin diseases, or allergic reactions to medications. Treatment is aimed at controlling bleeding episodes and addressing the underlying cause of the condition.

MalaCards based summary : Acquired Hemophilia a, also known as acquired factor viii deficiency disease, is related to hemophilia and factor vii deficiency. An important gene associated with Acquired Hemophilia a is F8 (Coagulation Factor VIII), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Collagen chain trimerization. The drugs Methylprednisolone and Methylprednisolone hemisuccinate have been mentioned in the context of this disorder. Affiliated tissues include myeloid, lung and kidney.

Related Diseases for Acquired Hemophilia a

Diseases in the Hemophilia family:

Hemophilia a Hemophilia B
Acquired Hemophilia Acquired Hemophilia a

Diseases related to Acquired Hemophilia a via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 216)
# Related Disease Score Top Affiliating Genes
1 hemophilia 31.9 F9 F8
2 factor vii deficiency 31.8 F9 F8 F3
3 compartment syndrome 30.6 F8 F3
4 hemophilia a 30.4 F9 F8A1 F8 F5 F3
5 acquired von willebrand syndrome 30.2 F8 F3
6 vitamin k deficiency bleeding 30.2 F9 F8 F3
7 hepatic veno-occlusive disease 30.1 F8 F3
8 acquired hemophilia 29.9 F9 F8A1 F8 F5 F3 F11
9 factor viii deficiency 29.9 F9 F8A1 F8 F5 F3 F11
10 hemopericardium 29.9 F9 F3
11 hemarthrosis 29.7 F9 F8 F3 F11
12 peripheral vascular disease 29.4 F5 F3
13 thrombophilia due to thrombin defect 29.3 F8 F5 F3
14 disseminated intravascular coagulation 29.2 F9 F5 F3
15 von willebrand's disease 29.1 F9 F8 F3 F11
16 hemorrhagic disease 29.0 F9 F8 F5 F3 F11
17 pulmonary embolism 28.9 F9 F8 F5 F3
18 thrombocytopenia 28.9 F9 F8 F5 F3 F11
19 thrombosis 28.5 F9 F8 F5 F3 F11
20 autoimmune disease 10.6
21 rare hemorrhagic disorder 10.5
22 pemphigoid 10.4
23 bullous pemphigoid 10.3
24 lupus erythematosus 10.3
25 purpura 10.3
26 systemic lupus erythematosus 10.2
27 myeloma, multiple 10.2
28 leukemia 10.2
29 thrombocytopenia due to platelet alloimmunization 10.2
30 hemolytic anemia 10.2
31 synovial angioma 10.1 F8 F3
32 leukemia, chronic lymphocytic 10.1
33 rheumatoid arthritis 10.1
34 anemia, autoimmune hemolytic 10.1
35 myasthenia gravis 10.1
36 hypothyroidism 10.1
37 pancreatitis 10.1
38 arthritis 10.1
39 pemphigus 10.1
40 diabetes mellitus 10.1
41 dysphagia 10.1
42 rare coagulation disorder 10.1
43 intracranial sinus thrombosis 10.1 F8 F3
44 angiodysplasia 10.1 F8 F3
45 multiple sclerosis 10.0
46 esophageal cancer 10.0
47 pemphigus vulgaris, familial 10.0
48 prostate cancer 10.0
49 lung cancer 10.0
50 myelofibrosis 10.0

Graphical network of the top 20 diseases related to Acquired Hemophilia a:



Diseases related to Acquired Hemophilia a

Symptoms & Phenotypes for Acquired Hemophilia a

Drugs & Therapeutics for Acquired Hemophilia a

Drugs for Acquired Hemophilia a (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 26)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
2
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
3
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
4
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
5
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
6
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
7
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
8
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
9
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
10 Methylprednisolone Acetate Phase 3
11 Alkylating Agents Phase 3
12 Immunoglobulins Phase 2, Phase 3
13 Antibodies Phase 2, Phase 3
14 Factor VIII Phase 2, Phase 3
15 Antirheumatic Agents Phase 2, Phase 3
16 Antineoplastic Agents, Immunological Phase 2, Phase 3
17 Immunologic Factors Phase 2, Phase 3
18 Hormones Phase 2, Phase 3
19 glucocorticoids Phase 2, Phase 3
20 Hormone Antagonists Phase 2, Phase 3
21 Antineoplastic Agents, Hormonal Phase 2, Phase 3
22 Anti-Inflammatory Agents Phase 2, Phase 3
23
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
24 Immunosuppressive Agents Phase 2
25 Autoantibodies
26 Coagulants

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Efficacy and Safety of B-Domain Deleted Recombinant Porcine Factor VIII (OBI-1) in the Treatment of Acquired Hemophilia A Due to Factor VIII Inhibitory Auto-antibodies Completed NCT01178294 Phase 2, Phase 3
2 A Prospective, Randomized, Multicenter Clinical Trial of Acquired Haemophilia A With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab Recruiting NCT03384277 Phase 3 Steroid;Rituximab;Cyclophosphamide
3 A Phase 2/3, Open-Label, Non-controlled Study to Evaluate the Efficacy and Safety of B-Domain Deleted Recombinant Porcine Factor VIII (rpFVIII, TAK-672), in the Treatment of Serious Bleeding Episode in Japanese Subjects With Acquired Hemophilia A (AHA) Not yet recruiting NCT04580407 Phase 2, Phase 3
4 A Prospective, Phase II/III Randomized, Mult-institutional Controlled, Open-label, Phase II Trial of Rituximab Versus Oral Cyclophosphamide to Eradicate or Suppress Autoimmune Anti-Factor VIII Antibodies in Patients With Acquired Hemophilia A Terminated NCT00306670 Phase 2, Phase 3 Rituxan;prednisone
5 An Open-label Phase II Study to Determine the Efficacy and Safety of Rituximab and Bortezomib in Patients With Newly Diagnosed Acquired Hemophilia A Unknown status NCT03700229 Phase 2 Bortezomib;Rituximab
6 Emicizumab in Patients With Acquired Hemophilia A: Multicenter, Single-arm, Open-label Clinical Trial Not yet recruiting NCT04188639 Phase 2 Emicizumab Injection
7 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Completed NCT02610127
8 Prospective and Retrospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
9 Acquired Hemophilia A and Autoimmunity. Study of Lymphocyte Populations and Myeloid-Derived Suppressor Cells Not yet recruiting NCT04805021
10 Expanded Access to B-Domain Deleted Recombinant Porcine Factor VIII (OBI-1) in the Treatment of Acquired Hemophilia A Due to Factor VIII Inhibitory Auto-antibodies No longer available NCT01968655

Search NIH Clinical Center for Acquired Hemophilia a

Genetic Tests for Acquired Hemophilia a

Genetic tests related to Acquired Hemophilia a:

# Genetic test Affiliating Genes
1 Acquired Factor Viii Deficiency Disease 29

Anatomical Context for Acquired Hemophilia a

MalaCards organs/tissues related to Acquired Hemophilia a:

40
Myeloid, Lung, Kidney, Liver, Prostate, Colon, Bone Marrow

Publications for Acquired Hemophilia a

Articles related to Acquired Hemophilia a:

(show top 50) (show all 399)
# Title Authors PMID Year
1
Hemostatic therapy as a management strategy for acquired hemophilia: what does the future hold? 61
33655810 2021
2
Transplacental hemophilia A and prophylactic treatment with intravenous immunoglobulin and recombinant factor VIIa in the newborn period: a case report. 61
33196507 2021
3
Should emicizumab be used in patients with acquired hemophilia A? 61
33306259 2021
4
Acquired Hemophilia A in Wiskott-Aldrich Syndrome. 61
33660143 2021
5
Acquired hemophilia A that required surgical hemostasis of hematomas occupying oral cavity: a case report. 61
33583426 2021
6
Case - Acquired hemophilia A in a patient with metastatic castration-resistant prostate cancer. 61
33591907 2021
7
Acquired Hemophilia A in an Elderly Patient on Apixaban Therapy. 61
33595223 2021
8
Ex Vivo Prediction of Comprehensive Coagulation Potential Using Simulated Blood Concentrations of Emicizumab in Patients with Acquired Hemophilia A. 61
33641138 2021
9
[Acquired hemophilia A: clinical and biological characteristics and therapeutic management of a series of eight patients hospitalized in Lariboisière and Saint-Louis hospitals]. 61
33622662 2021
10
Unforeseen encounter of acquired hemophilia A in a preoperative case of periampullary carcinoma: A case report. 61
33477072 2021
11
Idiopathic Acquired Hemophilia A, a Rare Cause of Bleeding: A Case Report and Literature Review. 61
33594037 2021
12
Acquired bleeding disorders. 61
32476241 2021
13
Principles of care for acquired hemophilia. 61
33527471 2021
14
Perioperative Bypassing Agent Therapy for Pulmonary Pleomorphic Carcinoma with Acquired Hemophilia. 61
33473052 2021
15
Treatment of acquired hemophilia A, a balancing act: results from a 27-year Dutch cohort study. 61
32974947 2021
16
Future directions in acquired hemophilia A. 61
33475739 2021
17
Emicizumab for the treatment of acquired hemophilia A. 61
32766881 2021
18
Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa: A case report. 61
33545998 2021
19
Acquired Hemophilia A and urothelial carcinoma. 61
33552425 2021
20
Successful Treatment of Acquired Hemophilia A with Bortezomib Plus Low Dose Rituximab in a Patient with Renal Failure and Severe Pneumonia. 61
33707854 2021
21
Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma. 61
33426985 2021
22
A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack. 61
32155618 2021
23
Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report. 61
33409062 2020
24
Recombinant porcine FVIII for bleed treatment in acquired hemophilia A: findings from a single-center, 18-patient cohort. 61
33351122 2020
25
Erratum: Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII? 61
33307578 2020
26
Diagnostic and Treatment Challenges for Acquired Hemophilia A in Pediatrics: Report of 2 Cases. 61
33181589 2020
27
[A case of acquired hemophilia A with multiple superficial blistering skin disorders as the main manifestation]. 61
33190449 2020
28
Acquired hemophilia A in a woman with systemic lupus erythematosus: A case report and review of literature. 61
33120848 2020
29
Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature. 61
33121522 2020
30
Acquired hemophilia A and other autoimmune diseases after alemtuzumab therapy for multiple sclerosis: A report of two cases. 61
32531751 2020
31
Undiagnosed Acquired Hemophilia A: Presenting as Recurrent Gastrointestinal Bleeding. 61
33029467 2020
32
Reduced-intensity, risk factor-stratified immunosuppression for acquired hemophilia A: single-center observational study. 61
32621181 2020
33
Surgery-associated acquired hemophilia A: a report of 2 cases and review of literature. 61
32967658 2020
34
Nuances in the management of acquired hemophilia A in an elderly patient with large granular lymphocytic leukemia. 61
32792468 2020
35
Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII? 61
32916754 2020
36
[Bullous pemphigoid associated with acquired hemophilia A]. 61
32008722 2020
37
Adalimumab-associated Acquired Hemophilia in a Patient with Scleritis. 61
32966141 2020
38
The first case of acquired hemophilia A associated with SARS-CoV-2 infection. 61
32401344 2020
39
Acquired Hemophilia A: A Case Report. 61
30727803 2020
40
International recommendations on the diagnosis and treatment of acquired hemophilia A. 61
32381574 2020
41
Bleeding and response to hemostatic therapy in acquired hemophilia A: results from the GTH-AH 01/2010 study. 61
32268359 2020
42
Pulmonary Embolism in Acquired Hemophilia A: A Rare Complication With Factor VIII Inhibitor Bypassing Activity Therapy. 61
32789089 2020
43
Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder. 61
32714682 2020
44
Plasma-derived factors VIIa and X mixtures (Byclot®) significantly improve impairment of coagulant potential ex vivo in plasmas from acquired hemophilia A patients. 61
32030609 2020
45
Postoperative hemorrhage secondary to acquired hemophilia A. 61
32452957 2020
46
Advances in managing rare acquired bleeding disorders. 61
32286895 2020
47
Utilization of Emicizumab in Acquired Factor VIII Deficiency. 61
32376817 2020
48
Acquired hemophilia A revealed by spontaneous bleeding in an 80-year-old man: a marginal diagnosis? 61
32459605 2020
49
Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission. 61
31939075 2020
50
An anti-factor IXa/factor X bispecific antibody, emicizumab, improves ex vivo coagulant potentials in plasma from patients with acquired hemophilia A. 61
31984625 2020

Variations for Acquired Hemophilia a

Expression for Acquired Hemophilia a

Search GEO for disease gene expression data for Acquired Hemophilia a.

Pathways for Acquired Hemophilia a

GO Terms for Acquired Hemophilia a

Cellular components related to Acquired Hemophilia a according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.65 F9 F8 F5 F3 F11
2 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.37 F8 F5
3 platelet alpha granule lumen GO:0031093 9.32 F8 F5
4 COPII-coated ER to Golgi transport vesicle GO:0030134 9.16 F8 F5
5 endoplasmic reticulum lumen GO:0005788 9.13 F9 F8 F5
6 extracellular space GO:0005615 9.02 F9 F8 F5 F3 F11

Biological processes related to Acquired Hemophilia a according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ER to Golgi vesicle-mediated transport GO:0006888 9.43 F9 F8 F5
2 platelet degranulation GO:0002576 9.37 F8 F5
3 blood coagulation GO:0007596 9.35 F9 F8 F5 F3 F11
4 blood coagulation, intrinsic pathway GO:0007597 9.33 F9 F8 F11
5 COPII vesicle coating GO:0048208 9.32 F8 F5
6 hemostasis GO:0007599 9.02 F9 F8 F5 F3 F11

Molecular functions related to Acquired Hemophilia a according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 copper ion binding GO:0005507 8.96 F8 F5
2 serine-type endopeptidase activity GO:0004252 8.8 F9 F3 F11

Sources for Acquired Hemophilia a

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....