Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034065 (pulmonary embolism)
 14,979 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired hemophilia A is a life-threatening immune-mediated hemorrhagic disorder that is most often found in individuals older than 50 who present with an unexplained activated partial thromboplastin time (aPTT) prolongation and clinically significant bleeding. The prolonged aPTT associated with acquired hemophilia A reflects factor VIII activity deficiency due to neutralizing or clearing autoantibodies. Deep venous thrombosis, in contrast, is a veno-occlusive disorder associated with several distinct hypercoagulable states that can result in significant morbidity and mortality due to pulmonary embolism, thrombus extension, and the post-thrombotic syndrome. A prolonged aPTT in the setting of thrombosis may reflect the presence of a lupus anticoagulant. In the absence of accurate diagnosis and the immediate institution of specific therapy, both disorders can be fatal. Three cases of acquired factor VIII inhibitors that included a prolonged aPTT, bleeding, and duplex ultrasound evidence of deep venous thrombosis are presented. The diagnostic and therapeutic challenges posed by these cases as well as a proposed mechanism by which pathologic thrombosis can develop in a patient with a life-threatening bleeding disorder are discussed.
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PMID:Simultaneous deep venous thrombosis and acquired factor VIII inhibitor. 1251 88

Acquired hemophilia A (AHA) is an uncommon complication caused by autoantibodies against Factor VIII. The main concern with these patients is hemorrhage, which is often treated with Factor VIII inhibitor bypassing activity (FEIBA). On rare occasions, treatment with FEIBA can result in thromboembolism, a potentially fatal complication. This unfortunate situation occurred in our patient, a 64-year-old female who was treated with FEIBA after being diagnosed with AHA. After initiating FEIBA, she developed clinical signs and symptoms of pulmonary embolism, which was ultimately responsible for her acute death. While pulmonary embolism may be a rare complication of FEIBA treatment, clinicians should be aware of its possibility, especially as the complete safety profile for this treatment is not well known.
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PMID:Pulmonary Embolism in Acquired Hemophilia A: A Rare Complication With Factor VIII Inhibitor Bypassing Activity Therapy. 3278 89