MCID: ACQ042
MIFTS: 37

Acquired Hemophilia a

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

Aliases & Classifications for Acquired Hemophilia a

MalaCards integrated aliases for Acquired Hemophilia a:

Name: Acquired Hemophilia a 52
Acquired Factor Viii Deficiency Disease 29
Acquired Factor Vii Deficiency Disease 71
Acquired Factor Vii Deficiency 52
Factor 8 Deficiency, Acquired 71
Acquired Factor 8 Deficiency 52
Acquired Hemophilia 71

Classifications:



External Ids:

UMLS 71 C0272321 C0272325 C1096116

Summaries for Acquired Hemophilia a

NIH Rare Diseases : 52 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 factor vii deficiency and hemophilia. 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 skin, lung and prostate.

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 203)
# Related Disease Score Top Affiliating Genes
1 factor vii deficiency 32.3 F9 F8 F3
2 hemophilia 31.7 F9 F8
3 hemophilia a 30.6 F9 F8A1 F8 F3
4 compartment syndrome 30.5 F8 F3
5 hepatic veno-occlusive disease 29.9 F8 F3
6 vitamin k deficiency bleeding 29.9 F9 F8 F3
7 hypothyroidism 29.5 F9 F8 F3
8 hemarthrosis 29.2 F9 F8 F3 F11
9 thrombocytopenia 29.1 F9 F5 F3
10 acquired hemophilia 29.0 F9 F8A1 F8 F5 F3 F11
11 acquired von willebrand syndrome 28.9 F9 F8 F3 F11
12 peripheral vascular disease 28.8 F5 F3
13 hemorrhagic disease 28.3 F9 F8 F5 F3 F11
14 disseminated intravascular coagulation 28.2 F9 F5 F3 F11
15 von willebrand's disease 27.8 F9 F8 F5 F3 F11
16 factor viii deficiency 27.8 F9 F8A1 F8 F5 F3 F11
17 autoimmune disease 10.6
18 rare hemorrhagic disorder 10.4
19 bullous pemphigoid 10.3
20 purpura 10.2
21 fournier gangrene 10.1 F8 F3
22 synovial angioma 10.1 F8 F3
23 alkhurma hemorrhagic fever 10.1 F8 F3
24 lupus erythematosus 10.1
25 alpha-2-plasmin inhibitor deficiency 10.1 F8 F3
26 angiodysplasia 10.1 F8 F3
27 von willebrand disease, type 1 10.1 F8 F3
28 systemic lupus erythematosus 10.1
29 myeloma, multiple 10.1
30 thrombocytopenia due to platelet alloimmunization 10.1
31 hemolytic anemia 10.1
32 multicentric castleman disease 10.1 F8 F3
33 mycoplasma pneumoniae pneumonia 10.1 F8 F3
34 brachydactyly, type d 10.1 F9 F8
35 posterior cerebral artery infarction 10.0 F9 F3
36 leukemia, chronic lymphocytic 10.0
37 anemia, autoimmune hemolytic 10.0
38 pancreatitis 10.0
39 pemphigus 10.0
40 dysphagia 10.0
41 rare coagulation disorder 10.0
42 blood platelet disease 10.0 F8 F3
43 high molecular weight kininogen deficiency 10.0 F3 F11
44 occlusion precerebral artery 10.0 F3 F11
45 esophageal cancer 9.9
46 pemphigus vulgaris, familial 9.9
47 rheumatoid arthritis 9.9
48 myasthenia gravis 9.9
49 myelofibrosis 9.9
50 leukemia, acute myeloid 9.9

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 phosphate Approved, Vet_approved Phase 3 302-25-0
4 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
5
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
6
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
7
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
8
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
9
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
10 Immunosuppressive Agents Phase 3
11 Methylprednisolone Acetate Phase 3
12 Alkylating Agents Phase 3
13 Immunoglobulins Phase 2, Phase 3
14 Antibodies Phase 2, Phase 3
15 Factor VIII Phase 2, Phase 3
16 Immunologic Factors Phase 2, Phase 3
17 Antirheumatic Agents Phase 2, Phase 3
18 Antineoplastic Agents, Immunological Phase 2, Phase 3
19 Hormones Phase 2, Phase 3
20 Antineoplastic Agents, Hormonal Phase 2, Phase 3
21 Hormone Antagonists Phase 2, Phase 3
22 glucocorticoids Phase 2, Phase 3
23 Anti-Inflammatory Agents Phase 2, Phase 3
24
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
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 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
4 An Open-label Phase II Study to Determine the Efficacy and Safety of Rituximab and Bortezomib in Patients With Newly Diagnosed Acquired Hemophilia A Recruiting NCT03700229 Phase 2 Bortezomib;Rituximab
5 Emicizumab in Patients With Acquired Hemophilia A: Multicenter, Single-arm, Open-label Clinical Trial Not yet recruiting NCT04188639 Phase 2 Emicizumab Injection
6 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Completed NCT02610127
7 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
8 Prospective and Retrospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
9 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
Skin, Lung, Prostate, Testes, Kidney, Heart, Colon

Publications for Acquired Hemophilia a

Articles related to Acquired Hemophilia a:

(show top 50) (show all 361)
# Title Authors PMID Year
1
Postoperative hemorrhage secondary to acquired hemophilia A. 61
32452957 2020
2
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
3
Acquired bleeding disorders. 61
32476241 2020
4
Advances in managing rare acquired bleeding disorders. 61
32286895 2020
5
International recommendations on the diagnosis and treatment of acquired hemophilia A. 61
32381574 2020
6
The first case of acquired hemophilia A associated with SARS-CoV-2 infection. 61
32401344 2020
7
Acquired hemophilia A revealed by spontaneous bleeding in an 80-year-old man: a marginal diagnosis? 61
32459605 2020
8
Utilization of Emicizumab in Acquired Factor VIII Deficiency. 61
32376817 2020
9
Acquired hemophilia A and other autoimmune diseases after alemtuzumab therapy for multiple sclerosis: A report of two cases. 61
32531751 2020
10
Bilateral simultaneous orbital hemorrhage and compartment syndrome as the presenting sign of acquired hemophilia A in an 11-year-old girl. 61
32061785 2020
11
Acquired Hemophilia A, Hemolytic Anemia, Type 1 Diabetes Mellitus, and Autoimmune Hypothyroidism in a Systemic Lupus Erythematosus Patient. 61
32251048 2020
12
Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission. 61
31939075 2020
13
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
14
A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review. 61
32284775 2020
15
Bleeding and Response to Hemostatic Therapy in Acquired Hemophilia A (AHA): Results from the GTH-AH 01/2010 Study. 61
32268359 2020
16
A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack. 61
32155618 2020
17
Airway Obstruction Caused by Hemorrhage: Managing a Life-Threatening Complication in Patients With Acquired Hemophilia A: A Case Report. 61
31842198 2020
18
Long-term outcomes in the treatment of acquired hemophilia A: a 16-year single institution prospective cohort experience. 61
31789659 2020
19
Cross-reacting inhibitors against recombinant porcine factor VIII in acquired hemophilia A: Data from the GTH-AH 01/2010 Study. 61
31448877 2020
20
[Acquired hemophilia A complicated by dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid]. 61
32507807 2020
21
An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria. 61
31929957 2020
22
Acquired hemophilia A. 61
32063777 2020
23
[Bullous pemphigoid associated with acquired hemophilia A]. 61
32008722 2020
24
Acquired Hemophilia A with Gastrointestinal Bleeding. 61
31280527 2020
25
Acquired factor VIII deficiency in prostate adenocarcinoma presenting as multiple hematomas and hemarthrosis. 61
32110407 2020
26
Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center. 61
31815811 2019
27
Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China. 61
31514689 2019
28
Management of rare acquired bleeding disorders. 61
31808848 2019
29
[Successful treatment of acquired hemophilia A with recombinant porcine factor VIII]. 61
29774375 2019
30
Esophageal Hematoma Mimicking a Large Esophageal Polyp: A Diagnostic Clue of Acquired Hemophilia A. 61
31585586 2019
31
Pharyngeal obstruction due to hemorrhagic bullae in a patient with anti-BP180-type mucous membrane pemphigoid associated with acquired hemophilia A. 61
31062391 2019
32
Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies. 61
31602421 2019
33
Safety and Efficacy of Rituximab and Cyclophosphamide in a Case of Resistant Acquired Hemophilia A in Course of Chronic Lymphocytic Leukemia. 61
31700733 2019
34
Acquired Hemophilia A: Three Cases and Review of the Literature. 61
31532083 2019
35
Acquired Hemophilia A After Nivolumab Therapy in a Patient With Metastatic Squamous Cell Carcinoma of the Lung Successfully Managed With Rituximab. 61
31311716 2019
36
Acquired hemophilia A in chronic lymphocytic leukemia: A case report. 61
31320278 2019
37
Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder. 61
31632887 2019
38
Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan. 61
31165409 2019
39
Acquired Hemophilia A Associated with NSAID: A Case Report. 61
31699950 2019
40
Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention. 61
31294330 2019
41
Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report. 61
31318850 2019
42
Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan. 61
30927215 2019
43
Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab. 61
30680669 2019
44
Bullous Pemphigoid Associated with Acquired Hemophilia A: A Case Report and Review of the Literature. 61
31182947 2019
45
Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report. 61
30882350 2019
46
Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report. 61
30864964 2019
47
Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients. 61
30958453 2019
48
[ACQUIRED HEMOPHILIA A AND THE TIMING OF IMMUNOMODULATORY THERAPY]. 61
30916502 2019
49
[Adequate diagnosis of acquired hemophilia A]. 61
31344171 2019
50
[PREGNANCY RELATED ACQUIRED HEMOPHILIA A]. 61
30916507 2019

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 9.55 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 9.26 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
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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