MCID: ACQ014
MIFTS: 51

Acquired Hemophilia

Categories: Rare diseases, Blood diseases, Genetic 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 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 skin, testes and lung, 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
Severe Hemophilia a

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

(show top 50) (show all 148)
# Related Disease Score Top Affiliating Genes
1 hemophilia 32.3 F3 F8 F9
2 acquired hemophilia a 30.3 F11 F3 F5 F8 F8A1 F9
3 hemarthrosis 29.5 F10 F8 F9
4 thrombosis 28.6 F10 F3 F5 F9
5 pulmonary embolism 28.1 F10 F3 F5 F9
6 hemophilia a 27.7 F10 F11 F3 F5 F8 F9
7 von willebrand's disease 26.9 F11 F3 F5 F8 F9
8 hemorrhagic disease 26.0 F10 F11 F3 F5 F8 F9
9 fournier gangrene 10.5 F3 F8
10 von willebrand disease, type 1 10.4 F3 F8
11 multicentric castleman disease 10.4 F3 F8
12 cardiac tamponade 10.4 F8 F9
13 mild hemophilia a 10.4 F10 F8
14 heparin-induced thrombocytopenia 10.3 F10 F3
15 leech infestation 10.3 F10 F3
16 hepatic veno-occlusive disease 10.3 F3 F8
17 carotid artery thrombosis 10.3 F10 F3
18 factor v and factor viii, combined deficiency of, 2 10.2 F5 F8
19 cerebral sinovenous thrombosis 10.2 F3 F5
20 retinal artery occlusion 10.2 F5 F8
21 sagittal sinus thrombosis 10.2 F3 F5
22 thrombophlebitis 10.1 F5 F8
23 arthritis 10.1
24 catastrophic antiphospholipid syndrome 10.1 F3 F5
25 carotid artery occlusion 10.1 F5 F8
26 rheumatoid arthritis 10.1
27 post-thrombotic syndrome 10.1 F5 F8
28 x-linked disease 10.1 F8 F9
29 peripheral vertigo 10.0 F3 F5
30 systemic lupus erythematosus 10.0
31 leukemia 10.0
32 lupus erythematosus 10.0
33 prothrombin deficiency, congenital 10.0 F10 F5
34 protein s deficiency 10.0 F3 F5
35 inherited blood coagulation disease 10.0 F3 F8 F9
36 bullous pemphigoid 10.0
37 glanzmann thrombasthenia 10.0 F3 F8 F9
38 thrombasthenia 10.0 F3 F5
39 immune system disease 9.9 F3 F8 F9
40 leukemia, chronic lymphocytic 2 9.9
41 leukemia, chronic lymphocytic 9.9
42 leukemia, b-cell, chronic 9.9
43 antithrombin iii deficiency 9.9 F10 F5
44 quebec platelet disorder 9.9 F10 F5
45 intracranial hypertension 9.9 F3 F5
46 scott syndrome 9.9 F10 F5
47 myeloma, multiple 9.9
48 diabetes mellitus 9.9
49 lymphoma 9.9
50 pancreatitis 9.9

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 subcutaneous hemorrhage 59 32 hallmark (90%) Very frequent (99-80%) HP:0001933
3 reduced factor viii activity 59 32 hallmark (90%) Very frequent (99-80%) HP:0003125
4 prolonged partial thromboplastin time 59 32 hallmark (90%) Very frequent (99-80%) HP:0003645
5 autoimmune antibody positivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0030057
6 intramuscular hematoma 59 32 frequent (33%) Frequent (79-30%) HP:0012233
7 hematuria 59 32 occasional (7.5%) Occasional (29-5%) HP:0000790
8 gastrointestinal hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0002239
9 post-partum hemorrhage 59 32 occasional (7.5%) Occasional (29-5%) HP:0011891
10 intracranial hemorrhage 59 32 very rare (1%) Very rare (<4-1%) HP:0002170
11 joint hemorrhage 59 32 very rare (1%) Very rare (<4-1%) HP:0005261
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 F9 F10 F11 F3 F5
2 homeostasis/metabolism MP:0005376 9.43 F5 F8 F9 F10 F11 F3
3 mortality/aging MP:0010768 9.1 F5 F8 F9 F10 F11 F3

Drugs & Therapeutics for Acquired Hemophilia

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

(show all 44)
# 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 Approved, Vet_approved Phase 3 50-24-8 5755
4
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
5
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
6 Factor VIII Phase 2, Phase 3,Phase 3
7 Immunosuppressive Agents Phase 3,Phase 2
8 Antirheumatic Agents Phase 3,Phase 2
9 Coagulants Phase 2, Phase 3,Phase 3
10 Antibodies Phase 2, Phase 3,Phase 3
11 Immunoglobulins Phase 2, Phase 3,Phase 3
12 Autoantibodies Phase 2, Phase 3
13 Prednisolone acetate Phase 3
14 Alkylating Agents Phase 3,Phase 2
15 glucocorticoids Phase 3,Phase 2
16 Methylprednisolone acetate Phase 3
17 Methylprednisolone Hemisuccinate Phase 3
18 Gastrointestinal Agents Phase 3
19 Neuroprotective Agents Phase 3
20 Hormone Antagonists Phase 3,Phase 2
21 Hormones Phase 3,Phase 2
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
23 Peripheral Nervous System Agents Phase 3
24 Prednisolone hemisuccinate Phase 3
25 Prednisolone phosphate Phase 3
26 Antiemetics Phase 3
27 Protective Agents Phase 3
28 Anti-Inflammatory Agents Phase 3,Phase 2
29 Autonomic Agents Phase 3
30 Antineoplastic Agents, Alkylating Phase 3,Phase 2
31 Antineoplastic Agents, Hormonal Phase 3,Phase 2
32
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
33
Azathioprine Approved 446-86-6 2265
34
Thrombin Approved, Investigational
35 Fibrinolytic Agents
36 Cyclosporins
37 Dermatologic Agents
38 Anticoagulants
39 Anti-Infective Agents
40 Antifungal Agents
41 Antimetabolites
42 Antimetabolites, Antineoplastic
43 Calcineurin Inhibitors
44 Hemostatics

Interventional clinical trials:

(show all 15)
# 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 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
6 Turkish Acquired Haemophilia Registry Unknown status NCT01403740 rFVIIIa, aPCC, FVIII, DDAVP, Azathioprine, Rituximab, Cyclosporin
7 Use of a TGA and TEM in the Assessment of the Efficacy of Treatment With APCC or rFVIIa Unknown status NCT01856751
8 Ex Vivo Laboratory Assays Application for Therapy Tailoring in Patients With Severe Bleeding Disorders Unknown status NCT02324517
9 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
10 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
11 Observational Study on the Efficacy and Safety of NovoSeven® During "Real-life" Usage in Germany Completed NCT00697320 eptacog alfa (activated)
12 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Recruiting NCT02610127
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 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, Testes, Lung, T Cells, Prostate, Neutrophil, B Cells

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 280)
# 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 After Hepatic Yttrium-90 Radioembolization: A Case Report. ( 28767473 )
2017
11
Autoimmune bullous disease and Hashimoto's disease complicated by acquired hemophilia A. ( 28381691 )
2017
12
Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report. ( 28870236 )
2017
13
Treatment of chronic lymphocytic leukemia/small lymphocytic lymphoma presenting simultaneously with acquired hemophilia and warm autoimmune hemolytic anemia. ( 28670081 )
2017
14
Treatment and complications in acquired hemophilia A. Experience from a single center. ( 28919213 )
2017
15
Acquired Hemophilia presenting as Gross Hematuria following Kidney Stone - A Case Report and Review of the Literature. ( 29144625 )
2017
16
Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan. ( 28299631 )
2017
17
Acquired hemophilia A: a review of recent data and new therapeutic options. ( 28441921 )
2017
18
Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature. ( 28458935 )
2017
19
Disappearance of acquired hemophilia A after complete remission in a Multiple Myeloma patient. ( 28077343 )
2017
20
Acquired Hemophilia A in a Patient with Non-Small Cell Lung Carcinoma: A Rare Paraneoplastic Phenomenon. ( 28457068 )
2017
21
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
22
Successful management of recurrent bleeding with tocilizumab in an acquired hemophilia A patient with rheumatoid arthritis. ( 28781267 )
2017
23
Acquired hemophilia A following generalized pustular psoriasis of pregnancy. ( 28771773 )
2017
24
Therapeutic plasma exchange as part of multimodal treatment of acquired hemophilia in a patient with concurrent acute intracerebral bleed and pulmonary embolism. ( 28436106 )
2017
25
Identification of people with acquired hemophilia in a large electronic health record database. ( 28769599 )
2017
26
Diagnosis and treatment of acquired hemophilia: aA single -center experience. ( 29112188 )
2017
27
Acquired hemophilia A in a patient treated by Ginkgo-dipyridamolum. ( 28174634 )
2017
28
Diagnosis and treatment of congenital and acquired hemophilia. ( 28781285 )
2017
29
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
30
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
31
Acquired hemophilia A: Updated review of evidence and treatment guidance. ( 28470674 )
2017
32
Acquired hemophilia as initial presentation in a patient with Systemic Lupus Erythematosus. ( 28523993 )
2017
33
Acquired Hemophilia and Anti-Hu Paraneoplastic Neurologic Syndrome in Small Cell Lung Cancer. ( 29078851 )
2017
34
Initial Manifestation of Acquired Hemophilia A After a Routine Tooth Extraction. A Case Report and Literature Review. ( 28886353 )
2017
35
An extra X does not prevent acquired hemophilia - Pregnancy-associated acquired hemophilia A. ( 28262242 )
2017
36
Acquired hemophilia A manifesting as plasma transfusion-uncontrolled severe bleeding 2 weeks after chorioamnionitis-induced abortion. ( 28150393 )
2017
37
Two elderly patients with difficult-to-treat acquired hemophilia A. ( 27885231 )
2016
38
Immunoglobulin G4-related acquired hemophilia: A case report. ( 28105131 )
2016
39
Myelofibrosis and acquired hemophilia A: a case report. ( 27154067 )
2016
40
Acquired hemophilia A in a patient with hepatocellular carcinoma: a case report and literature review. ( 27666283 )
2016
41
Coexistence of acquired hemophilia A and epidermolysis bullosa acquisita: Two case reports and published work review. ( 27511892 )
2016
42
Remission of acquired hemophilia A following radiation therapy for esophageal cancer. ( 27169449 )
2016
43
Unusual Initial Manifestation of Acquired Hemophilia A: A Normal Activated Partial Thromboplastin Time, Intramuscular Hematoma and Cerebral Hemorrhage. ( 27853081 )
2016
44
Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer. ( 27609734 )
2016
45
Bleeding due to acquired hemophilia A in acute pancreatitis: a case report. ( 26743559 )
2016
46
Acquired Hemophilia A May Be Associated with Ticagrelor Therapy in a 52-Year-Old Man After a Recent Percutaneous Transluminal Coronary Angioplasty. ( 27660505 )
2016
47
Acquired hemophilia A that developed during the induction of hemodialysis: the use of double-filtration plasmapheresisa8c. ( 27545905 )
2016
48
Use of recombinant activated factor VII for acute bleeding episodes in acquired hemophilia: final analysis from the Hemostasis and Thrombosis Research Society Registry acquired hemophilia study. ( 26761583 )
2016
49
The tipping point: The critical role of therapeutic apheresis in a case of refractory acquired hemophilia. ( 27646211 )
2016
50
Influence of factor VIII level and its inhibitor titer on the therapeutic response to corticosteroids alone in the management of acquired hemophilia: A retrospective single-center study. ( 27902587 )
2016

Variations for Acquired Hemophilia

Expression for Acquired Hemophilia

Search GEO for disease gene expression data for Acquired Hemophilia.

Pathways for Acquired Hemophilia

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 9.93 F10 F11 F3 F5 F8 F9
2 extracellular space GO:0005615 9.65 F11 F3 F5 F8 F9
3 extracellular region GO:0005576 9.63 F10 F11 F3 F5 F8 F9
4 Golgi lumen GO:0005796 9.43 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 9.62 F10 F5 F8 F9
2 blood coagulation, intrinsic pathway GO:0007597 9.46 F10 F11 F8 F9
3 COPII vesicle coating GO:0048208 9.43 F5 F8
4 blood coagulation, extrinsic pathway GO:0007598 9.43 F10 F3 F9
5 blood coagulation GO:0007596 9.43 F10 F11 F3 F5 F8 F9
6 signal peptide processing GO:0006465 9.4 F10 F9
7 peptidyl-glutamic acid carboxylation GO:0017187 9.37 F10 F9
8 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 copper ion binding GO:0005507 9.16 F5 F8
3 serine-type peptidase activity GO:0008236 9.13 F10 F11 F9
4 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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