PedAM

Acquired hemophilia is a rare autoimmune disorder characterized by bleeding that occurs in patients with a personal and family history negative for hemorrhages. Autoimmune disorders occur when the body's immune system mistakenly attacks healthy cells or tissue. In acquired hemophilia, the body produces antibodies (known as inhibitors) that attack clotting factors, most often factor VIII. Clotting factors are specialized proteins required for the blood to clot normally. Consequently, affected individuals develop complications associated with abnormal, uncontrolled bleeding into the muscles, skin and soft tissue and during surgery or following trauma. Specific symptoms can include nosebleeds (epistaxis), bruising throughout the body, solid swellings of congealed blood (hematomas), blood in the urine (hematuria) and gastrointestinal or urogenital bleeding. Acquired hemophilia can potentially cause severe, life-threatening bleeding complications in severe cases. In approximately 50% of cases, there is an identifiable underlying clinical condition; in the other 50% no cause is known (idiopathic).Acquired hemophilia is different from congenital hemophilia, a group of rare genetic disorders caused by congenital deficiency of certain clotting factors. The main form of hemophilia is hemophilia A (classic hemophilia), which is an X-linked disorder that fully affects males only. It is caused by deficiency or inactivation of factor VIII, the same clotting factor that is affected in most cases of acquired hemophilia. Although both disorders involve deficiency of the same clotting factor, the bleeding pattern is quite different. The reason the bleeding patterns differ between these disorders is not fully understood. - NORD
Reference: NORD

acquired haemophilia

C1096116

C0030805  |  bullous pemphigoid  |  2
C0003864  |  arthritis  |  1
C0024115  |  lung disease  |  1
C0409974  |  lupus erythematosus  |  1
C0026896  |  myasthenia gravis  |  1
C0001815  |  primary myelofibrosis  |  1
C0026986  |  myelodysplastic syndrome  |  1
C0024299  |  lymphoma  |  1
C0009492  |  compartment syndrome  |  1
C0003873  |  rheumatoid arthritis  |  1
C0035078  |  renal failure  |  1
C0019196  |  hepatitis c  |  1
C0019829  |  hodgkin lymphoma  |  1
C0024141  |  systemic lupus erythematosus  |  1
C0034150  |  purpura  |  1
C0001815  |  myelofibrosis  |  1
C0022660  |  acute renal failure  |  1
C0019158  |  hepatitis  |  1
C0030805  |  pemphigoid  |  1
C0376358  |  prostate cancer  |  1
C0035435  |  rheumatic disease  |  1
C0024305  |  non-hodgkin lymphoma  |  1
C0035435  |  rheumatic diseases  |  1
C0040028  |  essential thrombocythemia  |  1

HP:0003125  |  Reduced factor VIII activity
HP:0001903  |  Anemia
HP:0005261  |  Joint hemorrhage
HP:0002239  |  Gastrointestinal hemorrhage
HP:0011891  |  Post-partum hemorrhage
HP:0000790  |  Hematuria
HP:0002170  |  Intracranial hemorrhage
HP:0001892  |  Abnormal bleeding
HP:0030057  |  Autoimmune antibody positivity
HP:0001933  |  Subcutaneous hemorrhage
HP:0003645  |  Prolonged partial thromboplastin time
HP:0012233  |  Intramuscular hematoma

HP:0012189  |  Hodgkin disease  |  1
HP:0200123  |  Chronic liver inflammation  |  1
HP:0002863  |  Myelodysplastic syndrome  |  1
HP:0012125  |  Prostate cancer  |  1
HP:0000979  |  Purpura  |  1
HP:0003473  |  Fatigable weakness  |  1
HP:0002665  |  Lymphoma  |  1
HP:0002725  |  Systemic lupus erythematosus  |  1
HP:0001919  |  Acute renal failure  |  1
HP:0012539  |  Non-Hodgkin lymphoma  |  1
HP:0001369  |  Arthritis  |  1
HP:0001370  |  Rheumatoid arthritis  |  1
HP:0012115  |  Liver inflammation  |  1

C0085932  |  bullous dermatosis
C0085932  |  bullous dermatoses
C0030809  |  pemphigus vulgaris
C0019080  |  hemorrhage
C0018924  |  hemarthrosis
C0001883  |  airway obstruction

C0019080  |  hemorrhage  |  3