MCID: ACQ042
MIFTS: 44

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 54
Acquired Factor Viii Deficiency Disease 30
Acquired Factor Vii Deficiency Disease 74
Acquired Factor Vii Deficiency 54
Factor 8 Deficiency, Acquired 74
Acquired Factor 8 Deficiency 54
Acquired Hemophilia 74

Classifications:



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Summaries for Acquired Hemophilia a

NIH Rare Diseases : 54 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 rituximab and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and lung.

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 129)
# Related Disease Score Top Affiliating Genes
1 factor vii deficiency 32.3 F3 F8 F9
2 hemophilia 31.2 F3 F8 F9
3 factor viii deficiency 30.1 F11 F5 F8
4 hemophilia a 30.0 F11 F3 F5 F8 F9
5 hemarthrosis 29.8 F8 F9
6 acquired hemophilia 29.5 F11 F3 F5 F8 F8A1 F9
7 thrombosis 28.8 F3 F5 F8 F9
8 von willebrand's disease 28.3 F11 F3 F5 F8 F9
9 fournier gangrene 10.2 F3 F8
10 von willebrand disease, type 1 10.1 F3 F8
11 cardiac tamponade 10.1 F8 F9
12 myeloma, multiple 10.1
13 arthritis 10.1
14 von willebrand disease, type 2 10.1 F3 F8
15 rheumatoid arthritis 10.0
16 thrombocytopenia 10.0
17 thrombocytopenia due to platelet alloimmunization 10.0
18 pancreatitis 10.0
19 bullous pemphigoid 10.0
20 lemierre's syndrome 10.0 F11 F3
21 factor v and factor viii, combined deficiency of, 2 9.9 F5 F8
22 blood platelet disease 9.9 F3 F8
23 thrombasthenia 9.9 F3 F5
24 cerebral sinovenous thrombosis 9.9 F3 F5
25 thrombophlebitis 9.9 F5 F8
26 sagittal sinus thrombosis 9.9 F3 F5
27 systemic lupus erythematosus 9.9
28 prostate cancer 9.9
29 prostate cancer, hereditary, 8 9.9
30 prostate cancer, hereditary, 6 9.9
31 diabetes mellitus 9.9
32 leukemia 9.9
33 autoimmune pancreatitis 9.9
34 compartment syndrome 9.9
35 lupus erythematosus 9.9
36 pemphigus 9.9
37 carotid artery occlusion 9.9 F5 F8
38 catastrophic antiphospholipid syndrome 9.9 F3 F5
39 antiphospholipid syndrome 9.9 F3 F5
40 post-thrombotic syndrome 9.9 F5 F8
41 colorectal cancer 9.9
42 wilms tumor 5 9.9
43 leukemia, acute myeloid 9.9
44 wilms tumor 6 9.9
45 pyelonephritis 9.9
46 liposarcoma 9.9
47 myeloid leukemia 9.9
48 amyloidosis 9.9
49 al amyloidosis 9.9
50 inherited blood coagulation disease 9.9 F3 F8 F9

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 34)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 3,Phase 2 174722-31-7 10201696
2
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
3
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
4
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
6
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 6055-19-2, 50-18-0 2907
7
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
8
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
9 Factor VIII Phase 2, Phase 3,Phase 3
10 Immunoglobulins Phase 2, Phase 3,Phase 3
11 Coagulants Phase 2, Phase 3,Phase 3
12 Antibodies Phase 2, Phase 3,Phase 3
13 Autoantibodies Phase 2, Phase 3
14 Neuroprotective Agents Phase 3
15 Antirheumatic Agents Phase 3,Phase 2
16 Gastrointestinal Agents Phase 3
17 glucocorticoids Phase 3,Phase 2
18 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
19 Protective Agents Phase 3
20 Antineoplastic Agents, Hormonal Phase 3,Phase 2
21 Hormone Antagonists Phase 3,Phase 2
22 Immunologic Factors Phase 3,Phase 2
23 Alkylating Agents Phase 3,Phase 2
24 Antiemetics Phase 3
25 Methylprednisolone Acetate Phase 3
26 Anti-Inflammatory Agents Phase 3,Phase 2
27 Antineoplastic Agents, Alkylating Phase 3,Phase 2
28 Peripheral Nervous System Agents Phase 3
29 Prednisolone acetate Phase 3
30 Antineoplastic Agents, Immunological Phase 3,Phase 2
31 Hormones Phase 3,Phase 2
32 Autonomic Agents Phase 3
33 Immunosuppressive Agents Phase 3,Phase 2
34
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Study of Modified Recombinant Factor VIII (OBI-1) in Subjects With Acquired Hemophilia A Completed NCT01178294 Phase 2, Phase 3
2 Trial of Acquired Haemophilia With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab Recruiting NCT03384277 Phase 3 Steroid;Rituximab;Cyclophosphamide
3 Trial of Rituximab Versus Oral Cyclophosphamide to Eradicate or Suppress Autoimmune Anti-Factor VIII Antibodies in Acquired Hemophilia A Terminated NCT00306670 Phase 2, Phase 3 Rituxan;prednisone
4 Study to Evaluate Rituximab and Bortezomib in Patients With Newly Diagnosed Acquired Hemophilia A Recruiting NCT03700229 Phase 2 Bortezomib;Rituximab
5 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Recruiting NCT02610127
6 Prospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
7 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 30

Anatomical Context for Acquired Hemophilia a

MalaCards organs/tissues related to Acquired Hemophilia a:

42
Skin, Testes, Lung, Liver, Prostate, Bone, Bone Marrow

Publications for Acquired Hemophilia a

Articles related to Acquired Hemophilia a:

(show top 50) (show all 200)
# Title Authors Year
1
Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review. ( 30651844 )
2019
2
Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report. ( 30653138 )
2019
3
Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab. ( 30680669 )
2019
4
Acquired Hemophilia A: A Case Report. ( 30727803 )
2019
5
Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report. ( 30864964 )
2019
6
Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report. ( 30882350 )
2019
7
Subdural Hematoma From an Acquired Factor VII Deficiency: Is It the Lupus or the Anticoagulant? ( 30896459 )
2019
8
A Case of Acquired Hemophilia A: Usefulness of Various Methods for Judging Mixing Test Results for Monitoring the Effect of Immunosuppressive Therapy. ( 29739073 )
2018
9
The Development of Acute Systemic Multiple Thrombosis after Achieving Remission during Systemic Glucocorticoid Therapy for Acquired Hemophilia A. ( 29526954 )
2018
10
Can the plasmaderived factor VIII still play a role in the treatment of acquired hemophilia A at the time of new drugs? ( 29608457 )
2018
11
Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient. ( 29330401 )
2018
12
Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature. ( 29780157 )
2018
13
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
14
Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure. ( 29253693 )
2018
15
Acquired Hemophilia A: Experience of a Tertiary Care Institute from North India. ( 30127565 )
2018
16
A case of acquired hemophilia A in an elderly female. ( 30181835 )
2018
17
Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis: A case report and literature review. ( 30235659 )
2018
18
Disseminated microinfarctions of the right kidney requiring nephrectomy: an unusual complication of acquired hemophilia A. ( 30280718 )
2018
19
Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents. ( 30283617 )
2018
20
Management of acquired hemophilia A: Review of current evidence. ( 30396835 )
2018
21
Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series. ( 30502847 )
2018
22
Acquired hemophilia A in solid cancer: Two case reports and review of the literature. ( 30510943 )
2018
23
Management of Acquired Hemophilia A in Elderly Patients. ( 30538871 )
2018
24
Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A. ( 30542801 )
2018
25
Acquired Hemophilia A Presenting as Intramuscular Hematoma. ( 30574513 )
2018
26
A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury. ( 30627455 )
2018
27
Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver. ( 30344985 )
2018
28
Acquired Factor VII Deficiency in Association with Pyelonephritis. ( 30477072 )
2018
29
Acquired Hemophilia A After Hepatic Yttrium-90 Radioembolization: A Case Report. ( 28767473 )
2017
30
Autoimmune bullous disease and Hashimoto's disease complicated by acquired hemophilia A. ( 28381691 )
2017
31
Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report. ( 28870236 )
2017
32
Treatment and complications in acquired hemophilia A. Experience from a single center. ( 28919213 )
2017
33
Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan. ( 28299631 )
2017
34
Acquired hemophilia A: a review of recent data and new therapeutic options. ( 28441921 )
2017
35
Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature. ( 28458935 )
2017
36
Disappearance of acquired hemophilia A after complete remission in a Multiple Myeloma patient. ( 28077343 )
2017
37
Acquired Hemophilia A in a Patient with Non-Small Cell Lung Carcinoma: A Rare Paraneoplastic Phenomenon. ( 28457068 )
2017
38
First report of real-time monitoring of coagulation function potential and IgG subtype of anti-FVIII autoantibodies in a child with acquired hemophilia A associated with streptococcal infection and amoxicillin. ( 28597369 )
2017
39
Successful management of recurrent bleeding with tocilizumab in an acquired hemophilia A patient with rheumatoid arthritis. ( 28781267 )
2017
40
Acquired hemophilia A following generalized pustular psoriasis of pregnancy. ( 28771773 )
2017
41
Acquired hemophilia A in a patient treated by Ginkgo-dipyridamolum. ( 28174634 )
2017
42
Acquired hemophilia A resolution in a hepatitis C virus/human immunodeficiency virus-coinfected patient with cure of hepatitis C by direct-acting antiviral agents. ( 28960371 )
2017
43
Hemorrhagic pericardial effusion as the debut of acquired hemophilia in a chronic lymphocytic leukemia patient: A case report, and a review of acquired hemophilia A-related hematological malignancies. ( 29381944 )
2017
44
Acquired hemophilia A: Updated review of evidence and treatment guidance. ( 28470674 )
2017
45
Initial Manifestation of Acquired Hemophilia A After a Routine Tooth Extraction. A Case Report and Literature Review. ( 28886353 )
2017
46
An extra X does not prevent acquired hemophilia - Pregnancy-associated acquired hemophilia A. ( 28262242 )
2017
47
Acquired hemophilia A manifesting as plasma transfusion-uncontrolled severe bleeding 2 weeks after chorioamnionitis-induced abortion. ( 28150393 )
2017
48
Two elderly patients with difficult-to-treat acquired hemophilia A. ( 27885231 )
2016
49
Immunoglobulin G4-related acquired hemophilia: A case report. ( 28105131 )
2016
50
Myelofibrosis and acquired hemophilia A: a case report. ( 27154067 )
2016

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 F11 F3 F5 F8 F9
2 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.37 F5 F8
3 platelet alpha granule lumen GO:0031093 9.32 F5 F8
4 COPII-coated ER to Golgi transport vesicle GO:0030134 9.26 F5 F8
5 endoplasmic reticulum lumen GO:0005788 9.13 F5 F8 F9
6 extracellular space GO:0005615 9.02 F11 F3 F5 F8 F9

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 F5 F8 F9
2 platelet degranulation GO:0002576 9.37 F5 F8
3 blood coagulation GO:0007596 9.35 F11 F3 F5 F8 F9
4 blood coagulation, intrinsic pathway GO:0007597 9.33 F11 F8 F9
5 COPII vesicle coating GO:0048208 9.32 F5 F8
6 hemostasis GO:0007599 9.02 F11 F3 F5 F8 F9

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 F5 F8
2 serine-type endopeptidase activity GO:0004252 8.8 F11 F3 F9

Sources for Acquired Hemophilia a

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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