Acquired hemophilia: a rare but life-threatening potential cause of bleeding in the intensive care unit

Abstract

Recognition of acquired hemophilia presents a clinical challenge, given the rarity of this condition, a general lack of familiarity with acquired hemophilia, and the potential for confusion with other more common causes of bleeding in the intensive care unit. Nevertheless, there are sentinel clinical and laboratory findings that should raise suspicion of this diagnosis. The treatment of acquired hemophilia is a multi-step, physiologically focused process aimed at controlling both active and recurrent bleeding. Therefore, prompt diagnosis is central to prognosis. Consultation with a hematologist may facilitate efficient diagnosis and management.

The isolated prolonged PTT

“…11 If factor VIII activity is normal, measure factors IX and XI to assess for the rare possibility of a factor IX or XI inhibitor. 12 Acquired hemophilia must be suspected in elderly individuals with negative bleeding history who suddenly develop soft tissue hematomas and/or persistent and significant gastrointestinal or genitourinary hemorrhage [17]. This is a rare but serious autoimmune disorder, which can also occur in pregnancy or the postpartum period.…”

“…./Guidelines_Mng_Hemophilia.pdf. 17 A positive LA in a child under age 6 months is usually not considered clinically significant (unless it arises from the mother via transplacental antibody transfer) and the antibody is usually expected to disappear over a couple of weeks. However, exceptions can occur.…”

Aktuelle Gerinnungsdiagnostik in der Intensivmedizin

“…Präexistente angeborene Störungen im Bereich der Thrombingeneration sind selten und dem Patienten i. rungen im Bereich der Thrombingeneration ist die orale Antikoagulation mit Vitamin-K-Antagonisten wohl die häufigs-te Form, sehr selten hingegen sind die erworbenen "Hemmkörper" gegen einzelne Gerinnungsfaktoren -diese treten auch im höheren Alter auf und können die Ursache für lebensbedrohliche Blutungen sein [26]. Da die meisten präexistenten und unbekannten Gerinnungsstörungen im Bereich der primären Hämostase liegen, werden diese durch die klassischen Gerinnungsteste wie Quick und aPTT naturgemäß nicht erfaßt.…”

Acquired Hemophilia

“…Common bleeding sites include the muscles, skin, mucous membranes and soft tissue (1,2). Hemorrhage is often spontaneous and rarely follows trauma (1).…”