Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired factor VIII deficiency is a rare life-threatening disorder that should be suspected in individuals without a prior bleeding history who present with mucous membrane, muscle, and/or urinary tract bleeding. The authors describe a 5-year-old girl with epistaxis, intramuscular bleeding, and forearm compartment syndrome requiring emergent fasciotomy. Coagulation studies showed a factor VIII level of less than 1%. The prolonged activated partial thromboplastin time corrected immediately when mixed with normal plasma at a 1:1 ratio but became prolonged again following incubation at 37 degrees C. She was treated successfully with serial administrations of activated factor VII concentrates and immunosuppression with corticosteroids. Activated factor VII concentrates should be considered as an option for patients of all ages with acquired factor VIII deficiency.
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PMID:Successful treatment of acquired factor VIII deficiency in a child using activated factor VII concentrates: case report and review of the literature. 1284 30

Acquired factor VIII deficiency is very rare, often fatal. It is associated with pregnancy, autoimmune diseases, malignancy, and drugs, although no underlying cause is found in 50%. A 49-year-old male was referred with right shoulder bruising. The coagulation test showed a prolonged activated partial thromboplastin time. The factor VIII level was less than 1%, and the factor VIII inhibitor antibody titer was 246 Bethesda units/mL. The findings were compatible with acquired factor VIII deficiency. He had consumed the dried gallbladder of a cobra, Naja naja, for two weeks, it contained venom. After the initial treatment with factor VIII, he did not take supplemental coagulation factor VIII. The patient was readmitted with left forearm swelling. He lost consciousness suddenly and brain computed tomography (CT) revealed a subdural hematoma. Despite administering recombinant factor VII, his bleeding was not controlled and he died.
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PMID:Acquired factor VIII deficiency after consuming the dried gallbladder of a cobra, Naja naja. 2112 Feb 11

Acquired factor VIII deficiency is a rare disease that has high rates of mortality and morbidity. Although this bleeding disorder has a classic presentation, its infrequency can make it difficult to identify. We present a case report of an elderly woman who came to an emergency department with spontaneous bilateral lower extremity compartment syndrome. Although she presented with tachycardia and hypotension, her only laboratory abnormalities were an elevated activated partial thromboplastin time and anemia. She underwent bilateral fasciotomies and had a postoperative course that was complicated by significant incisional bleeding. She was ultimately found to have acquired factor VIII deficiency. Emergency physicians must be familiar with this rare entity because its recognition can avoid serious complications, particularly in those requiring operative management.
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PMID:Acquired factor VIII deficiency presenting with compartment syndrome. 2417 87