MCID: ACQ014
MIFTS: 52

Acquired Hemophilia

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

Aliases & Classifications for Acquired Hemophilia

MalaCards integrated aliases for Acquired Hemophilia:

Name: Acquired Hemophilia 53 59 73
Acquired Haemophilia 53

Characteristics:

Orphanet epidemiological data:

59
acquired hemophilia
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:



External Ids:

Orphanet 59 ORPHA73274
UMLS via Orphanet 74 C1096116
ICD10 via Orphanet 34 D68.4
UMLS 73 C1096116

Summaries for Acquired Hemophilia

NIH Rare Diseases : 53 Acquired hemophilia 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 occurs when the body's immune system attacks and disables a certain protein that helps the blood clot. 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, also known as acquired haemophilia, is related to acquired hemophilia a and hemophilia. An important gene associated with Acquired Hemophilia 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 Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and prostate, and related phenotypes are anemia and hematuria

Related Diseases for Acquired Hemophilia

Diseases in the Hemophilia family:

Hemophilia a Hemophilia B
Acquired Hemophilia Acquired Hemophilia a

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

(show top 50) (show all 171)
# Related Disease Score Top Affiliating Genes
1 acquired hemophilia a 33.0 F11 F3 F5 F8 F8A1 F9
2 hemophilia 32.5 F3 F8 F9
3 factor viii deficiency 30.3 F11 F5 F8
4 hemophilia a 30.2 F10 F11 F3 F5 F8 F9
5 hemarthrosis 29.9 F10 F8 F9
6 disseminated intravascular coagulation 29.6 F3 F5 F9
7 thrombosis 29.5 F10 F3 F5 F8 F9
8 pulmonary embolism 29.2 F10 F3 F5 F9
9 myocardial infarction 29.2 F10 F11 F3 F5 F8
10 von willebrand's disease 28.9 F11 F3 F5 F8 F9
11 factor v deficiency 28.6 F10 F11 F3 F5 F8 F9
12 arthritis 10.3
13 rheumatoid arthritis 10.2
14 systemic lupus erythematosus 10.1
15 lupus erythematosus 10.1
16 fournier gangrene 10.1 F3 F8
17 von willebrand disease, type 1 10.1 F3 F8
18 leukemia 10.1
19 bullous pemphigoid 10.1
20 cardiac tamponade 10.1 F8 F9
21 factor v and factor viii, combined deficiency of, 2 10.1 F5 F8
22 thrombasthenia 10.1 F3 F5
23 cerebral sinovenous thrombosis 10.1 F3 F5
24 sagittal sinus thrombosis 10.1 F3 F5
25 thrombophlebitis 10.1 F5 F8
26 catastrophic antiphospholipid syndrome 10.1 F3 F5
27 carotid artery occlusion 10.1 F5 F8
28 von willebrand disease, type 2 10.1 F3 F8
29 leukemia, chronic lymphocytic 2 10.1
30 leukemia, chronic lymphocytic 10.1
31 lymphocytic leukemia 10.1
32 leukemia, b-cell, chronic 10.1
33 post-thrombotic syndrome 10.1 F5 F8
34 leech infestation 10.0 F10 F3
35 heparin-induced thrombocytopenia 10.0 F10 F3
36 peripheral vertigo 10.0 F3 F5
37 blood protein disease 10.0 F3 F5
38 carotid artery thrombosis 10.0 F10 F3
39 protein s deficiency 10.0 F3 F5
40 myeloma, multiple 10.0
41 diabetes mellitus 10.0
42 lymphoma 10.0
43 pancreatitis 10.0
44 prothrombin deficiency, congenital 10.0 F10 F5
45 inherited blood coagulation disease 10.0 F3 F8 F9
46 intracranial hypertension 10.0 F3 F5
47 protein c deficiency 10.0 F5 F9
48 glanzmann thrombasthenia 10.0 F3 F8 F9
49 antithrombin iii deficiency 10.0 F10 F5
50 placenta disease 10.0 F3 F5

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



Diseases related to Acquired Hemophilia

Symptoms & Phenotypes for Acquired Hemophilia

Human phenotypes related to Acquired Hemophilia:

59 32 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 anemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001903
2 hematuria 59 32 occasional (7.5%) Occasional (29-5%) HP:0000790
3 gastrointestinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0002239
4 intracranial hemorrhage 59 32 very rare (1%) Very rare (<4-1%) HP:0002170
5 subcutaneous hemorrhage 59 32 hallmark (90%) Very frequent (99-80%) HP:0001933
6 prolonged partial thromboplastin time 59 32 hallmark (90%) Very frequent (99-80%) HP:0003645
7 autoimmune antibody positivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0030057
8 joint hemorrhage 59 32 very rare (1%) Very rare (<4-1%) HP:0005261
9 post-partum hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0011891
10 intramuscular hematoma 59 32 frequent (33%) Frequent (79-30%) HP:0012233
11 reduced factor viii activity 59 32 hallmark (90%) Very frequent (99-80%) HP:0003125
12 abnormal bleeding 59 Very frequent (99-80%)

MGI Mouse Phenotypes related to Acquired Hemophilia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.55 F10 F11 F3 F5 F9
2 homeostasis/metabolism MP:0005376 9.43 F10 F11 F3 F5 F8 F9
3 mortality/aging MP:0010768 9.1 F10 F11 F3 F5 F8 F9

Drugs & Therapeutics for Acquired Hemophilia

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

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 3,Phase 2 174722-31-7 10201696
2
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 6055-19-2, 50-18-0 2907
3
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
4
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
5
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
6
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
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 Immunologic Factors Phase 3,Phase 2
11 Antirheumatic Agents Phase 3,Phase 2
12 Immunosuppressive Agents Phase 3,Phase 2
13 Coagulants Phase 2, Phase 3,Phase 3
14 Autoantibodies Phase 2, Phase 3
15 Immunoglobulins Phase 2, Phase 3,Phase 3
16 Antibodies Phase 2, Phase 3,Phase 3
17 Antineoplastic Agents, Alkylating Phase 3,Phase 2
18 Alkylating Agents Phase 3,Phase 2
19 Methylprednisolone acetate Phase 3
20 Antineoplastic Agents, Hormonal Phase 3,Phase 2
21 Prednisolone acetate Phase 3
22 Anti-Inflammatory Agents Phase 3,Phase 2
23 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
24 Protective Agents Phase 3
25 Antiemetics Phase 3
26 Hormones Phase 3,Phase 2
27 Neuroprotective Agents Phase 3
28 Peripheral Nervous System Agents Phase 3
29 Hormone Antagonists Phase 3,Phase 2
30 Gastrointestinal Agents Phase 3
31 glucocorticoids Phase 3,Phase 2
32 Autonomic Agents Phase 3
33
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
34
Azathioprine Approved 446-86-6 2265
35
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
36
Thrombin Approved, Investigational
37 Antimetabolites, Antineoplastic
38 Dermatologic Agents
39 Antifungal Agents
40 Antimetabolites
41 Cyclosporins
42 Calcineurin Inhibitors
43 Anti-Infective Agents
44 Fibrinolytic Agents
45 Anticoagulants
46 Hemostatics

Interventional clinical trials:

(show all 16)
# 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 Outcome of Acquired Haemophilia With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab (CREHA Study) Recruiting NCT01808911 Phase 3 Steroid + cyclophosphamide;Steroid + rituximab
3 Trial of Acquired Haemophilia With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab Recruiting NCT03384277 Phase 3 Steroid;Rituximab;Cyclophosphamide
4 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
5 Study to Evaluate Rituximab and Bortezomib in Patients With Newly Diagnosed Acquired Hemophilia A Recruiting NCT03700229 Phase 2 Bortezomib;Rituximab
6 Bioequivalence of NovoSeven® and a NovoSeven® Formulation Stable at Room Temperature in Healthy Male Subjects Completed NCT01561417 Phase 1 activated recombinant human factor VII;activated recombinant human factor VII
7 Turkish Acquired Haemophilia Registry Unknown status NCT01403740 rFVIIIa, aPCC, FVIII, DDAVP, Azathioprine, Rituximab, Cyclosporin
8 Use of a TGA and TEM in the Assessment of the Efficacy of Treatment With APCC or rFVIIa Unknown status NCT01856751
9 Ex Vivo Laboratory Assays Application for Therapy Tailoring in Patients With Severe Bleeding Disorders Unknown status NCT02324517
10 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
11 Observational Study on the Use of NovoSeven® for Haemostatic Treatment of Bleeding Episodes in Patients With Acquired Haemophilia Completed NCT01285089 activated recombinant human factor VII
12 Observational Study on the Efficacy and Safety of NovoSeven® During "Real-life" Usage in Germany Completed NCT00697320 eptacog alfa (activated)
13 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Recruiting NCT02610127
14 Prospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
15 Global Haemostatic Methods Following Administration of Bypassing Agents to Patients With Haemophilia With Inhibitors Recruiting NCT02453542
16 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

Genetic Tests for Acquired Hemophilia

Anatomical Context for Acquired Hemophilia

MalaCards organs/tissues related to Acquired Hemophilia:

41
Skin, Lung, Prostate, Heart, Neutrophil, B Cells, Myeloid

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 518)
# Title Authors Year
1
A Case of Acquired Hemophilia A: Usefulness of Various Methods for Judging Mixing Test Results for Monitoring the Effect of Immunosuppressive Therapy. ( 29739073 )
2018
2
The Development of Acute Systemic Multiple Thrombosis after Achieving Remission during Systemic Glucocorticoid Therapy for Acquired Hemophilia A. ( 29526954 )
2018
3
Can the plasmaderived factor VIII still play a role in the treatment of acquired hemophilia A at the time of new drugs? ( 29608457 )
2018
4
Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient. ( 29330401 )
2018
5
Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature. ( 29780157 )
2018
6
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
7
Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure. ( 29253693 )
2018
8
Treatment of acquired hemophilia in the elderly. Study of 10 cases. ( 29685311 )
2018
9
Acquired hemophilia with thrombosis in a cancer patient: an unusual presentation. ( 29095762 )
2018
10
Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series. ( 30502847 )
2018
11
Management of Acquired Hemophilia A in Elderly Patients. ( 30538871 )
2018
12
Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A. ( 30542801 )
2018
13
Acquired Hemophilia in a Patient Presenting with Swollen Left Limb. ( 30001614 )
2018
14
Acquired Hemophilia Secondary to Soft-tissue Sarcoma: Case Report from a Latin American Hospital and Literature Review. ( 30027013 )
2018
15
Acquired Hemophilia of Unknown Etiology in an Elderly Man: Case Report. ( 30033442 )
2018
16
Acquired Hemophilia A: Experience of a Tertiary Care Institute from North India. ( 30127565 )
2018
17
A case of acquired hemophilia A in an elderly female. ( 30181835 )
2018
18
A Case Report of Anticoagulation Management in Acquired Hemophilia Associated With Levofloxacin. ( 30189787 )
2018
19
Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis: A case report and literature review. ( 30235659 )
2018
20
Disseminated microinfarctions of the right kidney requiring nephrectomy: an unusual complication of acquired hemophilia A. ( 30280718 )
2018
21
Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents. ( 30283617 )
2018
22
Management of acquired hemophilia A: Review of current evidence. ( 30396835 )
2018
23
Acquired hemophilia A in solid cancer: Two case reports and review of the literature. ( 30510943 )
2018
24
Acquired haemophilia A as a secondary autoimmune disease after alemtuzumab treatment in multiple sclerosis: A case report. ( 30513503 )
2018
25
Acquired haemophilia A imitating uterine tumour in a patient with de novo diagnosis of hepatitis C. ( 29953577 )
2018
26
Development of acquired haemophilia A in a patient treated with alemtuzumab for multiple sclerosis. ( 30337289 )
2018
27
Generalized pustular psoriasis as an unusual cause of acquired haemophilia A. ( 30105997 )
2018
28
Acquired haemophilia in cancer: A systematic and critical literature review. ( 28960809 )
2018
29
Laboratory monitoring issues in recombinant porcine FVIII replacement in acquired haemophilia A. ( 29418045 )
2018
30
A decade-long clinical experience on the prophylactic use of activated prothrombin complex concentrate in acquired haemophilia A: a case series from a tertiary care centre. ( 29474202 )
2018
31
Activated prothrombin complex concentrate (FEIBA® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry. ( 29528100 )
2018
32
Diagnostic delay in acquired haemophilia: Analysis of causes and consequences in a 20-year Spanish cohort. ( 29732654 )
2018
33
Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia. ( 29804265 )
2018
34
Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma. ( 30112740 )
2018
35
Retrospective review of Acquired Haemophilia A from the largest Canadian Haemophilia treatment centre. ( 30112783 )
2018
36
Correction to: Lessons from a systematic literature review of the effectiveness of recombinant factor VIIa in acquired haemophilia. ( 30267155 )
2018
37
Combined use of antifibrinolytics and activated prothrombin complex concentrate (aPCC) is not related to thromboembolic events in patients with acquired haemophilia A: data from FAIR Registry. ( 30267246 )
2018
38
Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A. ( 30473893 )
2018
39
aPCC vs. rFVIIa for the treatment of bleeding in patients with acquired haemophilia - a cost-effectiveness model. ( 30499154 )
2018
40
Acquired haemophilia A: a rare cause of postpartum haemorrhage. ( 30543618 )
2018
41
Low dose of aPCC after the initial treatment in acquired haemophilia A is useful to reduce bleeding relapses: Data from the FAIR registry. ( 30551040 )
2018
42
Acquired Hemophilia A After Hepatic Yttrium-90 Radioembolization: A Case Report. ( 28767473 )
2017
43
Autoimmune bullous disease and Hashimoto's disease complicated by acquired hemophilia A. ( 28381691 )
2017
44
Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report. ( 28870236 )
2017
45
Treatment of chronic lymphocytic leukemia/small lymphocytic lymphoma presenting simultaneously with acquired hemophilia and warm autoimmune hemolytic anemia. ( 28670081 )
2017
46
Treatment and complications in acquired hemophilia A. Experience from a single center. ( 28919213 )
2017
47
Acquired Hemophilia presenting as Gross Hematuria following Kidney Stone - A Case Report and Review of the Literature. ( 29144625 )
2017
48
Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan. ( 28299631 )
2017
49
Acquired hemophilia A: a review of recent data and new therapeutic options. ( 28441921 )
2017
50
Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature. ( 28458935 )
2017

Variations for Acquired Hemophilia

Expression for Acquired Hemophilia

Search GEO for disease gene expression data for Acquired Hemophilia.

Pathways for Acquired Hemophilia

Pathways related to Acquired Hemophilia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.81 F10 F11 F3 F5 F8 F9
2
Show member pathways
12.35 F10 F11 F3 F5 F8 F9
3 11.58 F10 F11 F3 F5 F8 F9
4
Show member pathways
11.4 F10 F9
5
Show member pathways
11.37 F10 F11 F3 F5 F8 F8A1
6 10.5 F10 F9

GO Terms for Acquired Hemophilia

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

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.91 F10 F11 F3 F5 F8 F9
2 extracellular region GO:0005576 9.73 F10 F11 F3 F5 F8 F9
3 extracellular space GO:0005615 9.63 F10 F11 F3 F5 F8 F9
4 Golgi lumen GO:0005796 9.46 F10 F9
5 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.4 F5 F8
6 platelet alpha granule lumen GO:0031093 9.37 F5 F8
7 COPII-coated ER to Golgi transport vesicle GO:0030134 9.32 F5 F8
8 endoplasmic reticulum lumen GO:0005788 9.26 F10 F5 F8 F9
9 intrinsic component of external side of plasma membrane GO:0031233 8.62 F10 F3

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

# Name GO ID Score Top Affiliating Genes
1 ER to Golgi vesicle-mediated transport GO:0006888 9.56 F10 F5 F8 F9
2 blood coagulation GO:0007596 9.43 F10 F11 F3 F5 F8 F9
3 COPII vesicle coating GO:0048208 9.37 F5 F8
4 blood coagulation, intrinsic pathway GO:0007597 9.33 F11 F8 F9
5 blood coagulation, extrinsic pathway GO:0007598 9.32 F10 F3
6 hemostasis GO:0007599 9.1 F10 F11 F3 F5 F8 F9

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

# Name GO ID Score Top Affiliating Genes
1 peptidase activity GO:0008233 9.43 F10 F11 F9
2 phospholipid binding GO:0005543 9.32 F10 F3
3 copper ion binding GO:0005507 9.16 F5 F8
4 serine-type peptidase activity GO:0008236 9.13 F10 F11 F9
5 serine-type endopeptidase activity GO:0004252 8.92 F10 F11 F3 F9

Sources for Acquired Hemophilia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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