MCID: ACQ014
MIFTS: 47

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 72
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:

ICD10 via Orphanet 34 D68.4
UMLS via Orphanet 73 C1096116
Orphanet 59 ORPHA73274
UMLS 72 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 Prednisolone phosphate and Methylprednisolone hemisuccinate have been mentioned in the context of this disorder. Affiliated tissues include skin, prostate 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

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

(show top 50) (show all 282)
# Related Disease Score Top Affiliating Genes
1 hemophilia 32.7 F9 F8 F3
2 acquired hemophilia a 31.5 F9 F8A1 F8 F5 F3 F11
3 factor viii deficiency 30.7 F8 F5 F11
4 hemarthrosis 30.1 F9 F8 F10
5 thrombasthenia 29.6 F5 F3
6 glanzmann thrombasthenia 29.6 F9 F8 F3
7 acquired von willebrand syndrome 29.6 F9 F8 F11
8 thrombophlebitis 29.6 F8 F5
9 disseminated intravascular coagulation 29.2 F9 F5 F3
10 factor vii deficiency 28.9 F9 F8 F3 F10
11 antiphospholipid syndrome 28.9 F5 F3 F10
12 hemophilia a 28.8 F9 F8 F5 F3 F11 F10
13 hemophilia b 28.5 F9 F8 F3 F11 F10
14 thrombophilia due to thrombin defect 28.4 F8 F5 F3 F10
15 pulmonary embolism 28.3 F9 F5 F3 F10
16 thrombosis 27.8 F9 F8 F5 F3 F10
17 von willebrand's disease 27.6 F9 F8 F5 F3 F11
18 myocardial infarction 27.5 F8 F5 F3 F11 F10
19 hemorrhagic disease 27.5 F9 F8 F5 F3 F11 F10
20 factor v deficiency 27.2 F9 F8 F5 F3 F11 F10
21 autoimmune disease 10.6
22 fournier gangrene 10.3 F8 F3
23 bullous pemphigoid 10.3
24 von willebrand disease, type 1 10.3 F8 F3
25 cardiac tamponade 10.3 F9 F8
26 lupus erythematosus 10.2
27 heparin-induced thrombocytopenia 10.2 F3 F10
28 systemic lupus erythematosus 10.2
29 rare hemorrhagic disorder 10.2
30 leech infestation 10.2 F3 F10
31 leukemia, chronic lymphocytic 10.2
32 lymphocytic leukemia 10.2
33 compartment syndrome 10.2
34 pemphigus 10.2
35 leukemia, b-cell, chronic 10.2
36 carotid artery thrombosis 10.2 F3 F10
37 von willebrand disease, type 2 10.2 F8 F3
38 rheumatoid arthritis 10.1
39 myeloma, multiple 10.1
40 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
41 acute kidney failure 10.1
42 purpura 10.1
43 anemia, autoimmune hemolytic 10.1
44 hemolytic anemia 10.1
45 pemphigus vulgaris, familial 10.0
46 leukemia, acute myeloid 10.0
47 respiratory failure 10.0
48 leukemia 10.0
49 thrombocytopenia due to platelet alloimmunization 10.0
50 thrombocytopenia 10.0

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 prolonged partial thromboplastin time 59 32 hallmark (90%) Very frequent (99-80%) HP:0003645
4 autoimmune antibody positivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0030057
5 reduced factor viii activity 59 32 hallmark (90%) Very frequent (99-80%) HP:0003125
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 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
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
2
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
3
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
4
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
5
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
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
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
9 Autoantibodies Phase 2, Phase 3
10 Gastrointestinal Agents Phase 3
11 Antiemetics Phase 3
12 Neuroprotective Agents Phase 3
13 Prednisolone acetate Phase 3
14 Methylprednisolone Acetate Phase 3
15 Peripheral Nervous System Agents Phase 3
16 Protective Agents Phase 3
17 Autonomic Agents Phase 3
18 Alkylating Agents Phase 2, Phase 3
19 Hormones Phase 2, Phase 3
20 glucocorticoids Phase 2, Phase 3
21 Immunologic Factors Phase 2, Phase 3
22 Anti-Inflammatory Agents Phase 2, Phase 3
23 Antineoplastic Agents, Immunological Phase 2, Phase 3
24 Antineoplastic Agents, Hormonal Phase 2, Phase 3
25 Immunosuppressive Agents Phase 2, Phase 3
26 Hormone Antagonists Phase 2, Phase 3
27 Factor VIII Phase 2, Phase 3
28 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2, Phase 3
29 Antirheumatic Agents Phase 2, Phase 3
30 Antineoplastic Agents, Alkylating Phase 2, Phase 3
31 Antibodies Phase 2, Phase 3
32 Coagulants Phase 2, Phase 3
33 Immunoglobulins Phase 2, Phase 3
34
Bortezomib Approved, Investigational Phase 2 179324-69-7 93860 387447
35
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
36
Azathioprine Approved 446-86-6 2265
37
Thrombin Approved, Investigational
38 Antifungal Agents
39 Anti-Infective Agents
40 Cyclosporins
41 Dermatologic Agents
42 Deamino Arginine Vasopressin
43 Antimetabolites, Antineoplastic
44 Calcineurin Inhibitors
45 Antimetabolites
46 Hemostatics

Interventional clinical trials:

(show all 14)
# 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 Outcome of Acquired Haemophilia With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab (CREHA Study) Recruiting NCT01808911 Phase 3 Steroid + cyclophosphamide;Steroid + rituximab
4 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
5 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
6 Observational 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 Concentrate in Patients With Acquired Haemophilia and in Patients With Haemophilia A With Inhibitors Unknown status NCT01856751
8 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
9 Prospective French Study on ACQUIred Haemophilia Treatment With Recombinant Factor VIIa (NovoSeven®) A Prospective Observational Study on the Use of NovoSeven® (Activated Recombinant Human Factor VIIa) for the Haemostatic Treatment of Bleeding Episodes in Patients With Acquired Haemophilia Completed NCT01285089 activated recombinant human factor VII
10 Efficacy of NovoSeven® (Activated Recombinant Human Factor VII): Non-interventional Study in Patients With Congenital Haemophilia With Inhibitors, Acquired Haemophilia, Factor FVII Deficiency, and Glanzmann's Thrombasthenia Completed NCT00697320 eptacog alfa (activated)
11 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Completed NCT02610127
12 Prospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
13 Global Haemostatic Methods to Measure the Treatment Effect Following Administration of Bypassing Agents to Patients With Haemophilia With Inhibitors Recruiting NCT02453542
14 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, Prostate, Lung, Testes, Kidney, T Cells, Liver

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 965)
# Title Authors PMID Year
1
[Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review]. 38
30904179 2019
2
Acquired Hemophilia A After Nivolumab Therapy in a Patient With Metastatic Squamous Cell Carcinoma of the Lung Successfully Managed With Rituximab. 38
31311716 2019
3
Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan. 38
31165409 2019
4
Outcome of CARE: a 6-year national registry of acquired haemophilia A in China. 38
31372978 2019
5
Epidemiology, spectrum of clinical manifestations and diagnostic issue of acquired haemophilia: A case series. 38
31427554 2019
6
B cell depletion therapy resulting in sustained remission of severe autoimmune complications following Alemtuzumab treatment of Multiple Sclerosis. 38
31357122 2019
7
New Hemostatic Agents: Perioperative Anesthetic Considerations. 38
31298165 2019
8
A Sardinian Family with Factor XI Deficiency. 38
31364091 2019
9
Acquired Hemophilia A with Gastrointestinal Bleeding. 38
31280527 2019
10
Acquired hemophilia A in chronic lymphocytic leukemia: A case report. 38
31320278 2019
11
Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report. 38
31318850 2019
12
Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention. 38
31294330 2019
13
Bidirectional graft-host hematological traffic in liver transplantation. 38
31245405 2019
14
Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris. 38
31183894 2019
15
Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan. 38
30927215 2019
16
Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab. 38
30680669 2019
17
Inhibitor eradication in refractory acquired hemophilia with lenalidomide. 38
30706086 2019
18
Acquired haemophilia A and concomitant factor XIII consumption. 38
30843631 2019
19
The factor VIII:C/VWF:Ag ratio as a useful tool to predict relapse in patients with acquired haemophilia A: A retrospective cohort study. 38
31050100 2019
20
Pharyngeal obstruction due to hemorrhagic bullae in a patient with anti-BP180-type mucous membrane pemphigoid associated with acquired hemophilia A. 38
31062391 2019
21
Systemic Lupus Erythematosus Presenting as Hematoma of the Hand Due to Acquired Inhibitors to Factor VIII: Early and Prolonged Remission Achieved with Upfront Rituximab. 38
31367504 2019
22
Bullous Pemphigoid Associated with Acquired Hemophilia A: A Case Report and Review of the Literature. 38
31182947 2019
23
Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report. 38
30882350 2019
24
Compartment syndrome of the forearm with life-threatening bleeding after fasciotomy as the presenting sign of postpartum acquired hemophilia A: a case report. 38
30864964 2019
25
Involvement of antifactor VIII autoantibodies specificity in the outcome of inhibitor eradication therapies in acquired hemophilia a patients. 38
30958453 2019
26
Emicizumab in the Treatment of Acquired Haemophilia: A Case Report. 38
31191199 2019
27
Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases. 38
30932442 2019
28
Activated prothrombin complex concentrate (FEIBA® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry. 38
29528100 2019
29
Relapse pattern and long-term outcomes in subjects with acquired haemophilia A. 38
30694571 2019
30
Acquired Hemophilia in a Patient Presenting with Swollen Left Limb. 38
30001614 2019
31
Treatment of acquired haemophilia with recombinant porcine factor VIII: A successful strategy in a patient with unstable angina. 38
30604899 2019
32
Expecting the unexpected: Acquired haemophilia A in a patient with homozygous factor V deficiency. 38
30690823 2019
33
[ACQUIRED HEMOPHILIA A AND THE TIMING OF IMMUNOMODULATORY THERAPY]. 38
30916502 2019
34
First Case of Acquired Hemophilia B in a Patient with HIV Infection: Case Report and Literature Review. 38
31106079 2019
35
[PREGNANCY RELATED ACQUIRED HEMOPHILIA A]. 38
30916507 2019
36
[Adequate diagnosis of acquired hemophilia A]. 38
31344171 2019
37
Acquired Hemophilia A: A Case Report. 38
30727803 2019
38
Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma. 38
30112740 2019
39
Thrombotic complications in adult patients with severe single coagulation factor or platelet defects - an overview. 38
30689456 2019
40
Low dose of aPCC after the initial treatment in acquired haemophilia A is useful to reduce bleeding relapses: Data from the FAIR registry. 38
30551040 2019
41
aPCC vs. rFVIIa for the treatment of bleeding in patients with acquired haemophilia - a cost-effectiveness model. 38
30499154 2019
42
[The issue of use parallelism factor method and index of circulating anticoagulant in diagnostic algorithm deficity of coagulation factors.] 38
30917248 2019
43
Treatment of acquired hemophilia in the elderly. Study of 10 cases. 38
29685311 2019
44
Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A. 38
30561877 2019
45
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. 38
30267246 2019
46
Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review. 38
30651844 2019
47
A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia. 38
30937199 2019
48
Bleeding Disorders Associated with Cancer. 38
31317489 2019
49
Usefulness of the APTT waveform for the diagnosis of DIC and prediction of the outcome or bleeding risk. 38
31297038 2019
50
Malignant benign hematology. 38
30656271 2019

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 F9 F8 F5 F3 F11 F10
2
Show member pathways
12.36 F9 F8 F5 F3 F11 F10
3 11.58 F9 F8 F5 F3 F11 F10
4
Show member pathways
11.4 F9 F10
5
Show member pathways
11.37 F9 F8A1 F8 F5 F3 F11
6 10.5 F9 F10

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

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 F9 F8 F5 F10
2 blood coagulation GO:0007596 9.43 F9 F8 F5 F3 F11 F10
3 COPII vesicle coating GO:0048208 9.37 F8 F5
4 blood coagulation, intrinsic pathway GO:0007597 9.33 F9 F8 F11
5 blood coagulation, extrinsic pathway GO:0007598 9.32 F3 F10
6 hemostasis GO:0007599 9.1 F9 F8 F5 F3 F11 F10

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 F9 F11 F10
2 phospholipid binding GO:0005543 9.32 F3 F10
3 copper ion binding GO:0005507 9.16 F8 F5
4 serine-type peptidase activity GO:0008236 9.13 F9 F11 F10
5 serine-type endopeptidase activity GO:0004252 8.92 F9 F3 F11 F10

Sources for Acquired Hemophilia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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