MCID: ACQ014
MIFTS: 45

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 52 58 71
Acquired Haemophilia 52

Characteristics:

Orphanet epidemiological data:

58
acquired hemophilia
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 D68.4
UMLS via Orphanet 72 C1096116
Orphanet 58 ORPHA73274
UMLS 71 C1096116

Summaries for Acquired Hemophilia

NIH Rare Diseases : 52 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 Methylprednisolone and Methylprednisolone hemisuccinate have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and thyroid, 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 290)
# Related Disease Score Top Affiliating Genes
1 hemophilia 33.1 F9 F8
2 acquired hemophilia a 31.9 F9 F8A1 F8 F5 F3 F11
3 hemophilia a 30.8 F9 F8A1 F8 F3
4 compartment syndrome 30.5 F8 F3
5 mycoplasma pneumoniae pneumonia 29.8 F8 F3
6 thrombasthenia 29.5 F5 F3
7 hypothyroidism 29.4 F9 F8 F3
8 thrombophlebitis 29.1 F8 F5 F3
9 acquired von willebrand syndrome 29.1 F9 F8 F3 F11
10 factor vii deficiency 28.9 F9 F8 F3 F10
11 glanzmann thrombasthenia 28.9 F9 F8 F3 F10
12 antiphospholipid syndrome 28.9 F5 F3 F10
13 hemarthrosis 28.8 F9 F8 F3 F11 F10
14 thrombocytopenia 28.8 F9 F5 F3 F10
15 thrombophilia due to thrombin defect 28.7 F8 F5 F3 F10
16 stroke, ischemic 28.7 F5 F3 F10
17 hemophilia b 28.7 F9 F8 F3 F11 F10
18 vascular disease 28.6 F8 F5 F3
19 factor viii deficiency 28.6 F9 F8A1 F8 F5 F3 F11
20 factor v deficiency 28.3 F9 F8 F5 F3 F10
21 pulmonary embolism 28.2 F9 F8 F5 F3 F10
22 disseminated intravascular coagulation 28.0 F9 F5 F3 F11 F10
23 hemorrhagic disease 27.9 F9 F8 F5 F3 F11 F10
24 von willebrand's disease 27.9 F9 F8 F5 F3 F11
25 myocardial infarction 27.5 F9 F8 F5 F3 F11 F10
26 thrombosis 27.5 F9 F8 F5 F3 F11 F10
27 autoimmune disease 10.6
28 rare hemorrhagic disorder 10.5
29 bullous pemphigoid 10.3
30 lupus erythematosus 10.2
31 leukemia, chronic lymphocytic 10.2
32 systemic lupus erythematosus 10.2
33 lymphocytic leukemia 10.2
34 fournier gangrene 10.2 F8 F3
35 synovial angioma 10.2 F8 F3
36 rheumatoid arthritis 10.2
37 pemphigus 10.2
38 angiodysplasia 10.2 F8 F3
39 von willebrand disease, type 1 10.1 F8 F3
40 myeloma, multiple 10.1
41 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
42 acute kidney failure 10.1
43 purpura 10.1
44 multicentric castleman disease 10.1 F8 F3
45 heparin-induced thrombocytopenia 10.1 F3 F10
46 anemia, autoimmune hemolytic 10.1
47 thrombocytopenia due to platelet alloimmunization 10.1
48 hemolytic anemia 10.1
49 leech infestation 10.1 F3 F10
50 carotid artery thrombosis 10.1 F3 F10

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:

58 31 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 anemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001903
2 subcutaneous hemorrhage 58 31 hallmark (90%) Very frequent (99-80%) HP:0001933
3 reduced factor viii activity 58 31 hallmark (90%) Very frequent (99-80%) HP:0003125
4 prolonged partial thromboplastin time 58 31 hallmark (90%) Very frequent (99-80%) HP:0003645
5 autoimmune antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030057
6 intramuscular hematoma 58 31 frequent (33%) Frequent (79-30%) HP:0012233
7 hematuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0000790
8 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
9 post-partum hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0011891
10 intracranial hemorrhage 58 31 very rare (1%) Very rare (<4-1%) HP:0002170
11 joint hemorrhage 58 31 very rare (1%) Very rare (<4-1%) HP:0005261
12 abnormal bleeding 58 Very frequent (99-80%)

Drugs & Therapeutics for Acquired Hemophilia

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

(show all 43)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
2
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
3
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
4 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
5
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
6
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
7
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
8
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
9
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
10 Autoantibodies Phase 2, Phase 3
11 Gastrointestinal Agents Phase 3
12 Antiemetics Phase 3
13 Methylprednisolone Acetate Phase 3
14 Protective Agents Phase 3
15 Autonomic Agents Phase 3
16 Neuroprotective Agents Phase 3
17 Alkylating Agents Phase 2, Phase 3
18 Antineoplastic Agents, Immunological Phase 2, Phase 3
19 Hormone Antagonists Phase 2, Phase 3
20 Immunosuppressive Agents Phase 2, Phase 3
21 Antirheumatic Agents Phase 2, Phase 3
22 Anti-Inflammatory Agents Phase 2, Phase 3
23 glucocorticoids Phase 2, Phase 3
24 Factor VIII Phase 2, Phase 3
25 Immunologic Factors Phase 2, Phase 3
26 Hormones Phase 2, Phase 3
27 Immunoglobulins Phase 2, Phase 3
28 Antibodies Phase 2, Phase 3
29 Coagulants Phase 2, Phase 3
30 Antineoplastic Agents, Hormonal Phase 2, Phase 3
31
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
32 Antibodies, Bispecific Phase 2
33
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
34
Azathioprine Approved 446-86-6 2265
35
Thrombin Approved, Investigational
36 Anti-Infective Agents
37 Dermatologic Agents
38 Deamino Arginine Vasopressin
39 Cyclosporins
40 Antifungal Agents
41 Antimetabolites
42 Calcineurin Inhibitors
43 Hemostatics

Interventional clinical trials:

(show all 15)
# 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 Emicizumab in Patients With Acquired Hemophilia A: Multicenter, Single-arm, Open-label Clinical Trial Not yet recruiting NCT04188639 Phase 2 Emicizumab Injection
7 Observational 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 Concentrate in Patients With Acquired Haemophilia and in Patients With Haemophilia A With Inhibitors Unknown status NCT01856751
9 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
10 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
11 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)
12 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Completed NCT02610127
13 Prospective and Retrospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
14 Global Haemostatic Methods to Measure the Treatment Effect 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:

40
Lung, Skin, Thyroid, Kidney, Prostate, T Cells, Liver

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 997)
# Title Authors PMID Year
1
Airway Obstruction Caused by Hemorrhage: Managing a Life-Threatening Complication in Patients With Acquired Hemophilia A: A Case Report. 61
31842198 2020
2
Plasma-derived factors VIIa and X mixtures (Byclot®) significantly improve impairment of coagulant potential ex vivo in plasmas from acquired hemophilia A patients. 61
32030609 2020
3
Magnesium-dependent activated partial thromboplastin time assay-Simple method for lupus anticoagulant detection. 61
31821738 2020
4
Acquired hemophilia. 61
32026881 2020
5
Patient profile, clinical characteristics and treatment response in acquired haemophilia A: A retrospective analysis of a single-centre experience over a 3-year period. 61
31742825 2020
6
An anti-factor IXa/factor X bispecific antibody, emicizumab, improves ex vivo coagulant potentials in plasma from patients with acquired hemophilia A. 61
31984625 2020
7
Acquired Hemophilia A with Gastrointestinal Bleeding. 61
31280527 2020
8
[Bullous pemphigoid associated with acquired hemophilia A]. 61
32008722 2020
9
An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria. 61
31929957 2020
10
Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission. 61
31939075 2020
11
Cross-reacting inhibitors against recombinant porcine factor VIII in acquired hemophilia A: Data from the GTH-AH 01/2010 Study. 61
31448877 2020
12
Long-term outcomes in the treatment of acquired hemophilia A: a 16-year single institution prospective cohort experience. 61
31789659 2020
13
Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China. 61
31514689 2019
14
Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center. 61
31815811 2019
15
Management of rare acquired bleeding disorders. 61
31808848 2019
16
Outcome of CARE: a 6-year national registry of acquired haemophilia A in China. 61
31372978 2019
17
[Oesophageal bullous detachment with massive haemorrhage revealing acquired haemophilia A in autoimmune bullous dermatosis]. 61
31831217 2019
18
An economic analysis of different treatments for bleeding in patients with acquired haemophilia. 61
31879973 2019
19
Clinical evaluation of bleeds and response to haemostatic treatment in patients with acquired haemophilia: A global expert consensus statement. 61
31517435 2019
20
Comment on 'Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris'. 61
31733157 2019
21
A Sardinian Family with Factor XI Deficiency. 61
31364091 2019
22
[Successful treatment of acquired hemophilia A with recombinant porcine factor VIII]. 61
29774375 2019
23
Esophageal Hematoma Mimicking a Large Esophageal Polyp: A Diagnostic Clue of Acquired Hemophilia A. 61
31585586 2019
24
Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris. 61
31183894 2019
25
Reply to 'Comment on "Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris"'. 61
31604364 2019
26
Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies. 61
31602421 2019
27
Pharyngeal obstruction due to hemorrhagic bullae in a patient with anti-BP180-type mucous membrane pemphigoid associated with acquired hemophilia A. 61
31062391 2019
28
B cell depletion therapy resulting in sustained remission of severe autoimmune complications following Alemtuzumab treatment of Multiple Sclerosis. 61
31357122 2019
29
Management of coagulation factor VIII (FVIII) inhibitors. 61
31477231 2019
30
The first reported case of acquired haemophilia A in which bleeding episodes were successfully treated via administration of a single-dose mixture of activated factor VIIa/X. 61
31487088 2019
31
Acquired Hemophilia A After Nivolumab Therapy in a Patient With Metastatic Squamous Cell Carcinoma of the Lung Successfully Managed With Rituximab. 61
31311716 2019
32
Safety and Efficacy of Rituximab and Cyclophosphamide in a Case of Resistant Acquired Hemophilia A in Course of Chronic Lymphocytic Leukemia. 61
31700733 2019
33
[Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review]. 61
30904179 2019
34
Acquired Hemophilia A: Three Cases and Review of the Literature. 61
31532083 2019
35
Acquired hemophilia A in chronic lymphocytic leukemia: A case report. 61
31320278 2019
36
Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder. 61
31632887 2019
37
Lupus anticoagulant-hypoprothrombinemia syndrome and similar diseases: experiences at a single center in Japan. 61
31165409 2019
38
[Post-transplantation lymphoproliferative disorder accompanies acquired hemophilia after haploid hematopoietic stem cell transplantation in a pediatric AML patient: a case report and literature review]. 61
31495141 2019
39
Epidemiology, spectrum of clinical manifestations and diagnostic issue of acquired haemophilia: A case series. 61
31427554 2019
40
Successful use of emicizumab in a patient with refractory acquired hemophilia A and acute coronary syndrome requiring percutaneous coronary intervention. 61
31294330 2019
41
Acquired Hemophilia A Associated with NSAID: A Case Report. 61
31699950 2019
42
Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report. 61
31318850 2019
43
Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab. 61
30680669 2019
44
Acquired Hemophilia A Associated with Dipeptidyl Peptidase-4 Inhibitors for the Treatment of Type 2 Diabetes Mellitus: A Single-Center Case Series in Japan. 61
30927215 2019
45
Bidirectional graft-host hematological traffic in liver transplantation. 61
31245405 2019
46
Inhibitor eradication in refractory acquired hemophilia with lenalidomide. 61
30706086 2019
47
Acquired haemophilia A and concomitant factor XIII consumption. 61
30843631 2019
48
The factor VIII:C/VWF:Ag ratio as a useful tool to predict relapse in patients with acquired haemophilia A: A retrospective cohort study. 61
31050100 2019
49
Systemic Lupus Erythematosus Presenting as Hematoma of the Hand Due to Acquired Inhibitors to Factor VIII: Early and Prolonged Remission Achieved with Upfront Rituximab. 61
31367504 2019
50
Acquired haemophilia A in a patient with breast cancer and lung carcinoma: a case report and literature review. 61
31502548 2019

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 extracellular region GO:0005576 9.73 F9 F8 F5 F3 F11 F10
2 extracellular space GO:0005615 9.63 F9 F8 F5 F3 F11 F10
3 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.4 F8 F5
4 platelet alpha granule lumen GO:0031093 9.37 F8 F5
5 COPII-coated ER to Golgi transport vesicle GO:0030134 9.32 F8 F5
6 endoplasmic reticulum lumen GO:0005788 9.26 F9 F8 F5 F10
7 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 copper ion binding GO:0005507 9.16 F8 F5
3 serine-type peptidase activity GO:0008236 9.13 F9 F11 F10
4 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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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