MCID: ACQ014
MIFTS: 51

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 54 60 74
Acquired Haemophilia 54

Characteristics:

Orphanet epidemiological data:

60
acquired hemophilia
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:



External Ids:

ICD10 via Orphanet 35 D68.4
UMLS via Orphanet 75 C1096116
Orphanet 60 ORPHA73274
UMLS 74 C1096116

Summaries for Acquired Hemophilia

NIH Rare Diseases : 54 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 hemophilia and acquired hemophilia a. 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 lung, skin and prostate, and related phenotypes are anemia and subcutaneous hemorrhage

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 184)
# Related Disease Score Top Affiliating Genes
1 hemophilia 32.6 F3 F8 F9
2 acquired hemophilia a 32.4 F11 F3 F5 F8 F8A1 F9
3 factor viii deficiency 30.8 F11 F5 F8
4 hemarthrosis 29.9 F10 F8 F9
5 hemophilia a 29.7 F10 F11 F3 F5 F8 F9
6 disseminated intravascular coagulation 29.2 F3 F5 F9
7 thrombosis 29.0 F10 F3 F5 F8 F9
8 pulmonary embolism 28.8 F10 F3 F5 F9
9 myocardial infarction 28.7 F10 F11 F3 F5 F8
10 hemophilia b 28.7 F10 F11 F3 F8 F9
11 von willebrand's disease 28.4 F11 F3 F5 F8 F9
12 factor v deficiency 28.2 F10 F11 F3 F5 F8 F9
13 arthritis 10.3
14 rheumatoid arthritis 10.2
15 fournier gangrene 10.2 F3 F8
16 lupus erythematosus 10.2
17 von willebrand disease, type 1 10.2 F3 F8
18 systemic lupus erythematosus 10.2
19 leukemia 10.2
20 bullous pemphigoid 10.2
21 cardiac tamponade 10.2 F8 F9
22 leech infestation 10.1 F10 F3
23 heparin-induced thrombocytopenia 10.1 F10 F3
24 carotid artery thrombosis 10.1 F10 F3
25 von willebrand disease, type 2 10.1 F3 F8
26 leukemia, chronic lymphocytic 2 10.1
27 leukemia, chronic lymphocytic 10.1
28 myeloma, multiple 10.1
29 diabetes mellitus 10.1
30 lymphocytic leukemia 10.1
31 leukemia, b-cell, chronic 10.1
32 thrombocytopenia 10.0
33 lymphoma 10.0
34 thrombocytopenia due to platelet alloimmunization 10.0
35 pancreatitis 10.0
36 lemierre's syndrome 10.0 F11 F3
37 inherited blood coagulation disease 10.0 F3 F8 F9
38 factor v and factor viii, combined deficiency of, 2 10.0 F5 F8
39 thrombasthenia 10.0 F3 F5
40 glanzmann thrombasthenia 10.0 F3 F8 F9
41 cerebral sinovenous thrombosis 10.0 F3 F5
42 autoimmune hepatitis 9.9
43 angioedema 9.9
44 dysphagia 9.9
45 sagittal sinus thrombosis 9.9 F3 F5
46 thrombophlebitis 9.9 F5 F8
47 catastrophic antiphospholipid syndrome 9.9 F3 F5
48 carotid artery occlusion 9.9 F5 F8
49 blood platelet disease 9.9 F10 F3 F8
50 post-thrombotic syndrome 9.9 F5 F8

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:

60 33 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 anemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0001903
2 subcutaneous hemorrhage 60 33 hallmark (90%) Very frequent (99-80%) HP:0001933
3 prolonged partial thromboplastin time 60 33 hallmark (90%) Very frequent (99-80%) HP:0003645
4 autoimmune antibody positivity 60 33 hallmark (90%) Very frequent (99-80%) HP:0030057
5 reduced factor viii activity 60 33 hallmark (90%) Very frequent (99-80%) HP:0003125
6 intramuscular hematoma 60 33 frequent (33%) Frequent (79-30%) HP:0012233
7 hematuria 60 33 occasional (7.5%) Occasional (29-5%) HP:0000790
8 gastrointestinal hemorrhage 60 33 occasional (7.5%) Occasional (29-5%) HP:0002239
9 post-partum hemorrhage 60 33 occasional (7.5%) Occasional (29-5%) HP:0011891
10 intracranial hemorrhage 60 33 very rare (1%) Very rare (<4-1%) HP:0002170
11 joint hemorrhage 60 33 very rare (1%) Very rare (<4-1%) HP:0005261
12 abnormal bleeding 60 Very frequent (99-80%)

MGI Mouse Phenotypes related to Acquired Hemophilia:

47
# 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 48)
# 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 50-18-0, 6055-19-2 2907
3
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
4
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
5
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
6
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
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 Antirheumatic Agents Phase 3,Phase 2
11 Antineoplastic Agents, Immunological Phase 3,Phase 2
12 Immunosuppressive Agents Phase 3,Phase 2
13 Immunologic Factors Phase 3,Phase 2
14 Coagulants Phase 2, Phase 3,Phase 3
15 Antibodies Phase 2, Phase 3,Phase 3
16 Autoantibodies Phase 2, Phase 3
17 Immunoglobulins Phase 2, Phase 3,Phase 3
18 Antineoplastic Agents, Alkylating Phase 3,Phase 2
19 Alkylating Agents Phase 3,Phase 2
20 Hormones Phase 3,Phase 2
21 Neuroprotective Agents Phase 3
22 Peripheral Nervous System Agents Phase 3
23 Antineoplastic Agents, Hormonal Phase 3,Phase 2
24 Gastrointestinal Agents Phase 3
25 Antiemetics Phase 3
26 glucocorticoids Phase 3,Phase 2
27 Anti-Inflammatory Agents Phase 3,Phase 2
28 Autonomic Agents Phase 3
29 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
30 Methylprednisolone Acetate Phase 3
31 Protective Agents Phase 3
32 Hormone Antagonists Phase 3,Phase 2
33 Prednisolone acetate Phase 3
34
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
35
Azathioprine Approved 446-86-6 2265
36
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
37
Thrombin Approved, Investigational
38 Dermatologic Agents
39 Antimetabolites, Antineoplastic
40 Cyclosporins
41 Antifungal Agents
42 Anti-Infective Agents
43 Calcineurin Inhibitors
44 Antimetabolites
45 Deamino Arginine Vasopressin
46 Fibrinolytic Agents
47 Anticoagulants
48 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 Prospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
14 Global Haemostatic Methods Following Administration of Bypassing Agents to Patients With Haemophilia With Inhibitors Recruiting NCT02453542
15 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Active, not recruiting NCT02610127
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:

42
Lung, Skin, Prostate, Thyroid, Myeloid, Testes, T Cells

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 589)
# Title Authors Year
1
Treatment of acquired hemophilia in the elderly. Study of 10 cases. ( 29685311 )
2019
2
Acquired Hemophilia in a Patient Presenting with Swollen Left Limb. ( 30001614 )
2019
3
Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review. ( 30651844 )
2019
4
Pregnancy-related acquired hemophilia A initially manifesting as pleural hemorrhage: A case report. ( 30653138 )
2019
5
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
6
Inhibitor eradication in refractory acquired hemophilia with lenalidomide. ( 30706086 )
2019
7
Acquired Hemophilia A: A Case Report. ( 30727803 )
2019
8
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
9
Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report. ( 30882350 )
2019
10
Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan. ( 30927215 )
2019
11
Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients. ( 30958453 )
2019
12
Pharyngeal obstruction due to hemorrhagic bullae in a patient with anti-BP180-type mucous membrane pemphigoid associated with acquired hemophilia A. ( 31062391 )
2019
13
First Case of Acquired Hemophilia B in a Patient with HIV Infection: Case Report and Literature Review. ( 31106079 )
2019
14
Expecting the unexpected: Acquired haemophilia A in a patient with homozygous factor V deficiency. ( 30690823 )
2019
15
The factor VIII:C/VWF:Ag ratio as a useful tool to predict relapse in patients with acquired haemophilia A: A retrospective cohort study. ( 31050100 )
2019
16
A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia. ( 30937199 )
2019
17
Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases. ( 30932442 )
2019
18
Acquired haemophilia A and concomitant factor XIII consumption. ( 30843631 )
2019
19
Relapse pattern and long-term outcomes in subjects with acquired haemophilia A. ( 30694571 )
2019
20
Treatment of acquired haemophilia with recombinant porcine factor VIII: A successful strategy in a patient with unstable angina. ( 30604899 )
2019
21
Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A. ( 30561877 )
2019
22
aPCC vs. rFVIIa for the treatment of bleeding in patients with acquired haemophilia - a cost-effectiveness model. ( 30499154 )
2019
23
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 )
2019
24
Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma. ( 30112740 )
2019
25
Activated prothrombin complex concentrate (FEIBA® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry. ( 29528100 )
2019
26
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 )
2018
27
Initial Manifestation of Acquired Hemophilia A After a Routine Tooth Extraction. A Case Report and Literature Review. ( 28886353 )
2018
28
Acquired hemophilia with thrombosis in a cancer patient: an unusual presentation. ( 29095762 )
2018
29
Acquired Hemophilia presenting as Gross Hematuria following Kidney Stone - A Case Report and Review of the Literature. ( 29144625 )
2018
30
Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure. ( 29253693 )
2018
31
Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient. ( 29330401 )
2018
32
The Development of Acute Systemic Multiple Thrombosis after Achieving Remission during Systemic Glucocorticoid Therapy for Acquired Hemophilia A. ( 29526954 )
2018
33
Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation. ( 29582334 )
2018
34
Can the plasmaderived factor VIII still play a role in the treatment of acquired hemophilia A at the time of new drugs? ( 29608457 )
2018
35
A Case of Acquired Hemophilia A: Usefulness of Various Methods for Judging Mixing Test Results for Monitoring the Effect of Immunosuppressive Therapy. ( 29739073 )
2018
36
Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature. ( 29780157 )
2018
37
Acquired Hemophilia Secondary to Soft-tissue Sarcoma: Case Report from a Latin American Hospital and Literature Review. ( 30027013 )
2018
38
Acquired Hemophilia of Unknown Etiology in an Elderly Man: Case Report. ( 30033442 )
2018
39
Acquired Hemophilia A: Experience of a Tertiary Care Institute from North India. ( 30127565 )
2018
40
A case of acquired hemophilia A in an elderly female. ( 30181835 )
2018
41
A Case Report of Anticoagulation Management in Acquired Hemophilia Associated With Levofloxacin. ( 30189787 )
2018
42
Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis: A case report and literature review. ( 30235659 )
2018
43
Disseminated microinfarctions of the right kidney requiring nephrectomy: an unusual complication of acquired hemophilia A. ( 30280718 )
2018
44
Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents. ( 30283617 )
2018
45
Management of acquired hemophilia A: Review of current evidence. ( 30396835 )
2018
46
Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series. ( 30502847 )
2018
47
Acquired hemophilia A in solid cancer: Two case reports and review of the literature. ( 30510943 )
2018
48
Management of Acquired Hemophilia A in Elderly Patients. ( 30538871 )
2018
49
Prevention and Management of Bleeding Episodes in Patients with Acquired Hemophilia A. ( 30542801 )
2018
50
Acquired Hemophilia A Presenting as Intramuscular Hematoma. ( 30574513 )
2018

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 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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