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 skin, lung 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 295)
# 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 hypothyroidism 29.6 F9 F8 F3
7 thrombasthenia 29.5 F5 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 thrombocytopenia 28.9 F9 F5 F3 F10
13 hemarthrosis 28.9 F9 F8 F3 F11 F10
14 antiphospholipid syndrome 28.9 F5 F3 F10
15 thrombophilia due to thrombin defect 28.8 F8 F5 F3 F10
16 hemophilia b 28.7 F9 F8 F3 F11 F10
17 vascular disease 28.7 F8 F5 F3
18 stroke, ischemic 28.4 F9 F5 F3 F10
19 factor v deficiency 28.3 F9 F8 F5 F3 F10
20 pulmonary embolism 28.2 F9 F8 F5 F3 F10
21 disseminated intravascular coagulation 28.0 F9 F5 F3 F11 F10
22 hemorrhagic disease 27.9 F9 F8 F5 F3 F11 F10
23 von willebrand's disease 27.9 F9 F8 F5 F3 F11
24 myocardial infarction 27.6 F9 F8 F5 F3 F11 F10
25 thrombosis 27.6 F9 F8 F5 F3 F11 F10
26 factor viii deficiency 27.4 F9 F8A1 F8 F5 F3 F11
27 autoimmune disease 10.6
28 rare hemorrhagic disorder 10.5
29 bullous pemphigoid 10.4
30 lupus erythematosus 10.3
31 systemic lupus erythematosus 10.2
32 leukemia, chronic lymphocytic 10.2
33 purpura 10.2
34 fournier gangrene 10.2 F8 F3
35 synovial angioma 10.2 F8 F3
36 alkhurma hemorrhagic fever 10.2 F8 F3
37 rheumatoid arthritis 10.2
38 pemphigus 10.2
39 alpha-2-plasmin inhibitor deficiency 10.1 F8 F3
40 acute kidney failure 10.1
41 hemolytic anemia 10.1
42 angiodysplasia 10.1 F8 F3
43 von willebrand disease, type 1 10.1 F8 F3
44 heparin-induced thrombocytopenia 10.1 F3 F10
45 posterior cerebral artery infarction 10.1 F9 F3
46 anemia, autoimmune hemolytic 10.1
47 myeloma, multiple 10.1
48 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
49 thrombocytopenia due to platelet alloimmunization 10.1
50 brachydactyly, type d 10.1 F9 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:

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 autoimmune antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030057
4 reduced factor viii activity 58 31 hallmark (90%) Very frequent (99-80%) HP:0003125
5 prolonged partial thromboplastin time 58 31 hallmark (90%) Very frequent (99-80%) HP:0003645
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 38)
# 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
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
7
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
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 Methylprednisolone Acetate Phase 3
12 Alkylating Agents Phase 3
13 Factor VIII Phase 2, Phase 3
14 Immunologic Factors Phase 2, Phase 3
15 Antirheumatic Agents Phase 2, Phase 3
16 Antineoplastic Agents, Immunological Phase 2, Phase 3
17 Antibodies Phase 2, Phase 3
18 Immunoglobulins Phase 2, Phase 3
19 Hormones Phase 2, Phase 3
20 Antineoplastic Agents, Hormonal Phase 2, Phase 3
21 Hormone Antagonists Phase 2, Phase 3
22 glucocorticoids Phase 2, Phase 3
23 Anti-Inflammatory Agents Phase 2, Phase 3
24
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
25 Immunosuppressive Agents Phase 2
26
Clotrimazole Approved, Vet_approved 23593-75-1 2812
27
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
28
Azathioprine Approved 446-86-6 2265
29
Thrombin Approved, Investigational
30 Deamino Arginine Vasopressin
31 Anti-Infective Agents
32 Antifungal Agents
33 Dermatologic Agents
34 Cyclosporins
35 Antimetabolites
36 Calcineurin Inhibitors
37 Hemostatics
38 Coagulants

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 Outcome of Acquired Haemophilia With Steroid Combined With Cyclophosphamide Versus Steroid Combined With Rituximab (CREHA Study) Unknown status NCT01808911 Phase 3 Steroid + cyclophosphamide;Steroid + rituximab
2 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
3 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
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
Skin, Lung, Prostate, Testes, Kidney, Thyroid, T Cells

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 1024)
# Title Authors PMID Year
1
A Case Report of Anticoagulation Management in Acquired Hemophilia Associated With Levofloxacin. 61
30189787 2020
2
Reply to 'Comment on "Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris"'. 61
31604364 2020
3
Comment on 'Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris'. 61
31733157 2020
4
Postoperative hemorrhage secondary to acquired hemophilia A. 61
32452957 2020
5
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
6
Acquired bleeding disorders. 61
32476241 2020
7
Advances in managing rare acquired bleeding disorders. 61
32286895 2020
8
Acquired Haemophilia A in DPP4 Inhibitor-induced Bullous Pemphigoid as Immune Reconstitution Syndrome. 61
32494825 2020
9
Susoctocog-alfa (Obizur®) in the treatment of nine elderly patients with acquired haemophilia A: an Italian multicentre real world experience. 61
32530407 2020
10
Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) - A case report. 61
32527615 2020
11
Acquired haemophilia A treated with recombinant porcine factor VIII: a single centre UK experience. 61
32141061 2020
12
​Acute airway compromise and coagulopathy: a rare presentation of acquired haemophilia A. 61
32371415 2020
13
Predictors of response and outcome of patients with acquired haemophilia A. 61
32469118 2020
14
International recommendations on the diagnosis and treatment of acquired hemophilia A. 61
32381574 2020
15
A hemophilia A mouse model for the in vivo assessment of Emicizumab function. 61
32369559 2020
16
The first case of acquired hemophilia A associated with SARS-CoV-2 infection. 61
32401344 2020
17
Acquired hemophilia A revealed by spontaneous bleeding in an 80-year-old man: a marginal diagnosis? 61
32459605 2020
18
Utilization of Emicizumab in Acquired Factor VIII Deficiency. 61
32376817 2020
19
Acquired hemophilia A and other autoimmune diseases after alemtuzumab therapy for multiple sclerosis: A report of two cases. 61
32531751 2020
20
Bilateral simultaneous orbital hemorrhage and compartment syndrome as the presenting sign of acquired hemophilia A in an 11-year-old girl. 61
32061785 2020
21
Acquired haemophilia A after alemtuzumab treatment of multiple sclerosis. 61
32301108 2020
22
Acquired Hemophilia A, Hemolytic Anemia, Type 1 Diabetes Mellitus, and Autoimmune Hypothyroidism in a Systemic Lupus Erythematosus Patient. 61
32251048 2020
23
Overshoot of FVIII activity in patients with acquired hemophilia A who achieve complete remission. 61
31939075 2020
24
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
25
Bleeding and Response to Hemostatic Therapy in Acquired Hemophilia A (AHA): Results from the GTH-AH 01/2010 Study. 61
32268359 2020
26
A Unique Presentation of Spontaneous Compartment Syndrome due to Acquired Hemophilia A and Associated Malignancy: Case Report and Literature Review. 61
32284775 2020
27
An economic analysis of different treatments for bleeding in patients with acquired haemophilia. 61
31879973 2020
28
[Oesophageal bullous detachment with massive haemorrhage revealing acquired haemophilia A in autoimmune bullous dermatosis]. 61
31831217 2020
29
A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack. 61
32155618 2020
30
Airway Obstruction Caused by Hemorrhage: Managing a Life-Threatening Complication in Patients With Acquired Hemophilia A: A Case Report. 61
31842198 2020
31
Acquired haemophilia and haemostatic control with recombinant porcine FVIII: case series. 61
32043744 2020
32
Magnesium-dependent activated partial thromboplastin time assay-Simple method for lupus anticoagulant detection. 61
31821738 2020
33
Acquired hemophilia. 61
32026881 2020
34
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
35
Long-term outcomes in the treatment of acquired hemophilia A: a 16-year single institution prospective cohort experience. 61
31789659 2020
36
Cross-reacting inhibitors against recombinant porcine factor VIII in acquired hemophilia A: Data from the GTH-AH 01/2010 Study. 61
31448877 2020
37
An Interesting Case of Acquired Hemophilia A in an Elderly Patient Presenting with Hematuria. 61
31929957 2020
38
[Acquired hemophilia A complicated by dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid]. 61
32507807 2020
39
Acquired Hemophilia A with Gastrointestinal Bleeding. 61
31280527 2020
40
Acquired hemophilia A. 61
32063777 2020
41
[Bullous pemphigoid associated with acquired hemophilia A]. 61
32008722 2020
42
Acquired factor VIII deficiency in prostate adenocarcinoma presenting as multiple hematomas and hemarthrosis. 61
32110407 2020
43
Acquired Hemophilia in an Elderly Patient with Carcinoma of the Ampulla of Vater. 61
32110212 2020
44
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
45
Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China. 61
31514689 2019
46
Management of rare acquired bleeding disorders. 61
31808848 2019
47
Outcome of CARE: a 6-year national registry of acquired haemophilia A in China. 61
31372978 2019
48
Clinical evaluation of bleeds and response to haemostatic treatment in patients with acquired haemophilia: A global expert consensus statement. 61
31517435 2019
49
A Sardinian Family with Factor XI Deficiency. 61
31364091 2019
50
[Successful treatment of acquired hemophilia A with recombinant porcine factor VIII]. 61
29774375 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
Content
Loading form....