Acquired hemophilia: Case report of severe hemorrhagic diathesis in a 72 year old hispanic

Matos-Fernandez, N.; Ortiz-Munoz, L.; Garcia, A.; Adorno-Fontanez, J.; Caceres, W.

Boletin de la Asociacion Medica de Puerto Rico 97(2): 144-150

2005


ISSN/ISBN: 0004-4849
Document Number: 703044
A 72-year-old man with seizure disorder and hypertension developed gross hematuria. The patient was found lethargic, hypotensive, with gross hematuria and multiple hematomas. Recent seizure episodes and trauma were denied. Laboratory results showed a prolonged aPTT, normal PT, low hemoglobin and hematocrit. The patient was treated with fresh frozen plasma with minimal improvement of his bleeding and without correction of the PTT. Several blood transfusions were required for optimization of hemoglobin levels. A mixing test was done with no correction of the PTT suggesting the presence of a factor inhibitor. The activity of factor VIII was 0% and levels of factor VIII inhibitors were as high as 525.9 BU. The patient received multiple treatments with resolution of the bleeding. The patient responded to rituximab, a monoclonal antibody against CD20, and melphalan illustrating the efficacy of those medications in regulating the production of auto-antibodies.

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Acquired hemophilia: Case report of severe hemorrhagic diathesis in a 72 year old hispanic