血液疾患と輸血ジャーナル

概要

Spontaneous Life Threatening Massive Retroperitoneal and Iliopsoas Hemorrhages in Adult Hemophilia - A Patients with Inhibitor - Case Series

Najmaddin Khoshnaw, Belal A. Muhammad and Ahmed k. Yassin

Spontaneous retroperitoneal and iliopsoas muscle hemorrhages are rare bleeding episodes in hemophilia patients. Their diagnosis requires high degree of clinical suspicion because presentation may mimic other abdominal and pelvic disorders. These bleedings in severe hemophilia-A patients with factor VIII inhibitor are associated with high morbidity and mortality as control of hemostasis in these cases is challenging.

Herein we report 4 adult patients with known congenital Hemophilia-A and factor VIII inhibitor. One of the patients had gigantic retroperitoneal hemorrhage presented with subsequent increase in abdominal pain. The other three patients had significant iliopsoas hemorrhage presented with groin pain. All the events were spontaneous and nontraumatic. These patients were treated with recombinant activated factor VII (rFVIIa) in a dose of 90 μg/kg. Treatment with rFVIIa has significant favorable outcome and all the patients had dramatic response to the treatment and discharged safely from the hospital after 7-10 days of admission. Patients with iliopsoas hemorrhage showed persistent femoral nerve neuropathy. The rarity and spontaneous bleeding episodes at presentation are crucial points for reporting these cases.