MCID: ACQ014
MIFTS: 45

Acquired Hemophilia

Categories: Blood diseases, Genetic diseases, Immune diseases, Muscle diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Acquired Hemophilia

MalaCards integrated aliases for Acquired Hemophilia:

Name: Acquired Hemophilia 20 58 70
Acquired Haemophilia 20

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 71 C1096116
Orphanet 58 ORPHA73274
UMLS 70 C1096116

Summaries for Acquired Hemophilia

GARD : 20 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, myeloid 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 305)
# Related Disease Score Top Affiliating Genes
1 hemophilia 32.4 F9 F8
2 acquired hemophilia a 30.9 F9 F8A1 F8 F5 F3 F11
3 compartment syndrome 30.5 F8 F3
4 hemophilia a 30.5 F9 F8A1 F8 F5 F3
5 acquired von willebrand syndrome 30.1 F8 F3
6 hemopericardium 30.1 F9 F3
7 thrombasthenia 29.6 F5 F3
8 factor viii deficiency 29.5 F9 F8A1 F8 F5 F3 F11
9 hemarthrosis 29.4 F9 F8 F3 F11 F10
10 thrombophlebitis 29.4 F8 F5 F3
11 factor vii deficiency 29.2 F9 F8 F3 F10
12 glanzmann thrombasthenia 1 29.2 F9 F8 F3 F10
13 antiphospholipid syndrome 29.2 F5 F3 F10
14 hemophilia b 29.2 F9 F8 F3 F11 F10
15 thrombophilia due to thrombin defect 29.1 F8 F5 F3 F10
16 von willebrand's disease 29.1 F9 F8 F3 F11
17 vascular disease 29.1 F8 F5 F3
18 disseminated intravascular coagulation 29.1 F9 F5 F3 F10
19 pulmonary embolism 28.9 F9 F8 F5 F3 F10
20 factor v deficiency 28.8 F9 F8 F5 F3 F10
21 thrombosis 28.6 F9 F8 F5 F3 F11 F10
22 hemorrhagic disease 28.6 F9 F8 F5 F3 F11 F10
23 thrombocytopenia 28.6 F9 F8 F5 F3 F11 F10
24 myocardial infarction 28.3 F9 F8 F5 F3 F11 F10
25 thrombophilia 28.1 F9 F8 F5 F3 F11 F10
26 autoimmune disease 10.6
27 rare hemorrhagic disorder 10.5
28 pemphigoid 10.4
29 bullous pemphigoid 10.4
30 lupus erythematosus 10.3
31 systemic lupus erythematosus 10.3
32 rheumatoid arthritis 10.3
33 arthritis 10.3
34 leukemia, chronic lymphocytic 10.2
35 purpura 10.2
36 myeloma, multiple 10.2
37 leukemia 10.2
38 pemphigus 10.2
39 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
40 acute kidney failure 10.2
41 hemolytic anemia 10.2
42 synovial angioma 10.2 F8 F3
43 intracranial sinus thrombosis 10.1 F8 F3
44 anemia, autoimmune hemolytic 10.1
45 lymphoma 10.1
46 hypothyroidism 10.1
47 thrombocytopenia due to platelet alloimmunization 10.1
48 angiodysplasia 10.1 F8 F3
49 heparin-induced thrombocytopenia 10.1 F3 F10
50 leech infestation 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 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 37)
# 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 Approved, Vet_approved Phase 3 50-24-8 5755
4
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
5
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
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 Methylprednisolone Acetate Phase 3
11 Alkylating Agents Phase 3
12 Antineoplastic Agents, Immunological Phase 2, Phase 3
13 Antirheumatic Agents Phase 2, Phase 3
14 Immunologic Factors Phase 2, Phase 3
15 Factor VIII Phase 2, Phase 3
16 Immunoglobulins Phase 2, Phase 3
17 Hormones Phase 2, Phase 3
18 glucocorticoids Phase 2, Phase 3
19 Hormone Antagonists Phase 2, Phase 3
20 Antineoplastic Agents, Hormonal Phase 2, Phase 3
21 Antibodies Phase 2, Phase 3
22 Anti-Inflammatory Agents Phase 2, Phase 3
23
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
24 Immunosuppressive Agents Phase 2
25
Clotrimazole Approved, Vet_approved 23593-75-1 2812
26
Miconazole Approved, Investigational, Vet_approved 22916-47-8 4189
27
Azathioprine Approved 446-86-6 2265
28 Cyclosporins
29 Deamino Arginine Vasopressin
30 Antifungal Agents
31 Anti-Infective Agents
32 Calcineurin Inhibitors
33 Antimetabolites
34 Dermatologic Agents
35 Hemostatics
36 Autoantibodies
37 Coagulants

Interventional clinical trials:

(show all 16)
# 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 Phase 2/3, Open-Label, Non-controlled Study to Evaluate the Efficacy and Safety of B-Domain Deleted Recombinant Porcine Factor VIII (rpFVIII, TAK-672), in the Treatment of Serious Bleeding Episode in Japanese Subjects With Acquired Hemophilia A (AHA) Not yet recruiting NCT04580407 Phase 2, Phase 3
5 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
6 An Open-label Phase II Study to Determine the Efficacy and Safety of Rituximab and Bortezomib in Patients With Newly Diagnosed Acquired Hemophilia A Unknown status NCT03700229 Phase 2 Bortezomib;Rituximab
7 Emicizumab in Patients With Acquired Hemophilia A: Multicenter, Single-arm, Open-label Clinical Trial Not yet recruiting NCT04188639 Phase 2 Emicizumab Injection
8 Observational Acquired Haemophilia Registry Unknown status NCT01403740 rFVIIIa, aPCC, FVIII, DDAVP, Azathioprine, Rituximab, Cyclosporin
9 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
10 Prospective Clinical and Biological Follow-up of Acquired Haemophilia : SACHA Study Completed NCT00213473
11 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
12 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)
13 Post-Marketing Non-Interventional Safety Evaluation of Obizur in the Treatment of Bleeding Episodes for Patients With Acquired Hemophilia A Completed NCT02610127
14 Prospective and Retrospective, Non-interventional Study to Evaluate the Safety and Effectiveness of Obizur in Real-life Practice Recruiting NCT03199794
15 Acquired Hemophilia A and Autoimmunity. Study of Lymphocyte Populations and Myeloid-Derived Suppressor Cells Not yet recruiting NCT04805021
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:

40
Lung, Myeloid, Prostate, Kidney, Liver, Thyroid, Whole Blood

Publications for Acquired Hemophilia

Articles related to Acquired Hemophilia:

(show top 50) (show all 1100)
# Title Authors PMID Year
1
Assessment of Lebanese healthcare professionals' awareness on acquired haemophilia: a cross-sectional study. 61
33427128 2021
2
Hematological immune related adverse events after treatment with immune checkpoint inhibitors. 61
33706206 2021
3
Onset of acquired haemophilia A after omalizumab treatment in severe bullous pemphigoid - a report on two cases successfully treated with mycophenolate mofetil. 61
33461795 2021
4
De novo acquired hemophilia as an immune dysregulation phenomenon following SARS-CoV-2 infection. 61
33368293 2021
5
Potential Clues to the Pathogenesis of Acquired Hemophilia. 61
33647996 2021
6
Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review. 61
33550699 2021
7
Hemostatic therapy as a management strategy for acquired hemophilia: what does the future hold? 61
33655810 2021
8
A Case Report of Acquired Haemophilia following COVID-19 Vaccine. 61
33783953 2021
9
Autoimmune Complications in Hematologic Neoplasms. 61
33810369 2021
10
Transplacental hemophilia A and prophylactic treatment with intravenous immunoglobulin and recombinant factor VIIa in the newborn period: a case report. 61
33196507 2021
11
Should emicizumab be used in patients with acquired hemophilia A? 61
33306259 2021
12
Acquired Hemophilia A in Wiskott-Aldrich Syndrome. 61
33660143 2021
13
Acquired hemophilia A that required surgical hemostasis of hematomas occupying oral cavity: a case report. 61
33583426 2021
14
Case - Acquired hemophilia A in a patient with metastatic castration-resistant prostate cancer. 61
33591907 2021
15
Acquired Hemophilia A in an Elderly Patient on Apixaban Therapy. 61
33595223 2021
16
Ex Vivo Prediction of Comprehensive Coagulation Potential Using Simulated Blood Concentrations of Emicizumab in Patients with Acquired Hemophilia A. 61
33641138 2021
17
Idiopathic Acquired Hemophilia A, a Rare Cause of Bleeding: A Case Report and Literature Review. 61
33594037 2021
18
[Acquired hemophilia A: clinical and biological characteristics and therapeutic management of a series of eight patients hospitalized in Lariboisière and Saint-Louis hospitals]. 61
33622662 2021
19
Unforeseen encounter of acquired hemophilia A in a preoperative case of periampullary carcinoma: A case report. 61
33477072 2021
20
Acquired bleeding disorders. 61
32476241 2021
21
Principles of care for acquired hemophilia. 61
33527471 2021
22
Acquired haemophilia and haemostatic control with recombinant porcine factor VIII: case series. 61
32043744 2021
23
Acquired hemophilia in a 7-year-old girl successfully treated with recombinant FVIIA and steroids: A case report. 61
33598217 2021
24
Immunoadsorption for the Treatment of Acquired Hemophilia: New Observational Data, Systematic Review, and Meta-Analysis. 61
33518429 2021
25
Bullous pemphigoid is a common associated disorder with acquired haemophilia A. 61
32949372 2021
26
Sudden Death of a Young Hemophiliac by Low-Velocity Blunt Knee Trauma in Bullock Cart Run-Over Fatality. 61
33585112 2021
27
Perioperative Bypassing Agent Therapy for Pulmonary Pleomorphic Carcinoma with Acquired Hemophilia. 61
33473052 2021
28
Paraneoplastic acquired haemophilia A in extensive-stage small cell lung cancer (ES-SCLC) in the era of immunotherapy. 61
33509862 2021
29
Plasma Exchange as an Initial Treatment for Severe Bleeding Induced by Acquired Factor V Deficiency: A Case Report and Mini Literature Review. 61
32784304 2021
30
Two case reports of acquired haemophilia A as complications of alemtuzumab treatment for multiple sclerosis. 61
33681807 2021
31
Acquired hemophilia A and delta storage pool deficiency in a patient with indolent non-Hodgkin lymphoma. 61
33426985 2021
32
Treatment of acquired hemophilia A, a balancing act: results from a 27-year Dutch cohort study. 61
32974947 2021
33
Future directions in acquired hemophilia A. 61
33475739 2021
34
Emicizumab for the treatment of acquired hemophilia A. 61
32766881 2021
35
Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa: A case report. 61
33545998 2021
36
Acquired Hemophilia A and urothelial carcinoma. 61
33552425 2021
37
Successful Treatment of Acquired Hemophilia A with Bortezomib Plus Low Dose Rituximab in a Patient with Renal Failure and Severe Pneumonia. 61
33707854 2021
38
A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack. 61
32155618 2021
39
Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report. 61
33409062 2020
40
Recombinant porcine FVIII for bleed treatment in acquired hemophilia A: findings from a single-center, 18-patient cohort. 61
33351122 2020
41
Erratum: Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII? 61
33307578 2020
42
Bilateral renal haematuria and obstructive renal failure from acquired haemophilia A: a medical cause for a surgical problem. 61
33334751 2020
43
Persistent inhibitor in acquired haemophilia A: A case for emicizumab? 61
33314498 2020
44
The Art of Detecting Antibodies against Factor VIII. 61
32961576 2020
45
Acquired haemophilia A after alemtuzumab therapy. 61
32700327 2020
46
Acquired Haemophilia. 61
33187043 2020
47
Clinical utility of sample preheat treatment in a modified Nijmegen-Bethesda assay (mNBA) for inhibitor monitoring in congenital and acquired haemophilia A: A single-centre four-year experience. 61
32892451 2020
48
Cross-reacting recombinant porcine FVIII inhibitors in patients with acquired haemophilia A. 61
32997894 2020
49
Diagnostic and Treatment Challenges for Acquired Hemophilia A in Pediatrics: Report of 2 Cases. 61
33181589 2020
50
Acquired haemophilia A, concomitant acute myocardial infarction and urgent major surgery: How to successfully treat a critical patient with rpFVIII (Obizur®). 61
32683151 2020

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 Golgi lumen GO:0005796 9.43 F9 F10
4 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 9.4 F8 F5
5 platelet alpha granule lumen GO:0031093 9.37 F8 F5
6 COPII-coated ER to Golgi transport vesicle GO:0030134 9.32 F8 F5
7 endoplasmic reticulum lumen GO:0005788 9.26 F9 F8 F5 F10
8 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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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