Thrombotic thrombocytopenic purpura: a rare cause of thrombocytopenia in HIV-infected hemophiliacs.

Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophiliacs. The etiology is multifactorial and a majority of the patients with hemophilia exhibit a decreased platelet count within 10 years of seroconversion. Thrombocytopenia in these patients is associated with a high risk of bleeding and death. Thrombotic microangiopathy causing thrombocytopenia in HIV-infected hemophiliacs is extremely rare. We describe an HIV-infected hemophilic patient who presented with bleeding, renal insufficiency, and thrombocytopenia. Platelet transfusion resulted in deterioration of clinical condition. Examination of blood smears demonstrated a microangiopathic process. The patient responded well to plasmapheresis with normalization of platelet and renal function. Thrombotic thrombocytopenic purpura should be suspected in HIV-infected hemophiliacs who present with a new onset of thrombocytopenia and anemia as delay in treatment may result in fatal sequelae.

Controlled prospective study of factor IX concentrate therapy and immunodeficiency.

In 1983, six patients who were exclusively treated with factor IX (FIX) concentrate (greater than 40,000 units/yr) were prospectively matched for age and dosage to six patients treated exclusively with factor VIII (FVIII) concentrate and to six normal male controls. At baseline evaluation between October 1983 and May 1984, both groups had significantly decreased absolute T helper cell counts (mean of 452/microliters and 505/microliters for FIX- and FVIII-treated groups, respectively) compared to normal (1,157/microliters). By August 1988, three of the six FIX-treated group have developed AIDS and two are seropositive for antibody to the human immunodeficiency virus (HIV). Four of the six FVIII-treated group have HIV seropositivity or disease. The other three patients (1/6 for FIX and 2/6 for FVIII) declined HIV antibody testing. Our results support other studies showing a dose-related risk of HIV exposure for FIX concentrate-treated patients and do not support the view that FIX concentrate was intrinsically safer than FVIII concentrate.

A consortium for purchase of blood products directed by physicians.

Clotting-factor therapy is a costly part of comprehensive hemophilia treatment. Physicians treating patients with hemophilia in New York formed a consortium for the purchasing and regional distribution of clotting-factor concentrates. Concentrates are centrally purchased based on a bidding formula aimed at obtaining the lowest price for quality product while guaranteeing all suppliers continued involvement in a large market area. The consortium has successfully maintained, and, in instances, lowered prices each year. Estimated regional savings this year are more than $750,000 for an anticipated purchase of approximately 60,00,000 units of clotting-factors VIII and IX. Central distribution has additionally lowered costs to participating hospitals. The consortium is able to provide patients and third-party payers with some of the lowest prices for clotting-factor concentrates in the United States. A physician-directed regional approach to purchasing costly medical products might be applied to other areas of clinical medicine.

Severe von Willebrand's disease during labor and delivery.

In pregnant women with severe von Willebrand's disease, the prepartum period should include monitoring for a rise in factor VIII-related activities (factor VIII coagulant activity [factor VIII:C], factor VIII-related antigen [VIII R:Ag], and factor VIII ristocetin cofactor [factor VIII R:Cof]). Two women whose pregnancies were so monitored were delivered of normal infants: one by cesarean section and one by vaginal delivery. The failure of factor VIII:Ag or factor VIII R:Cof to rise above the level of 50% at term in the woman who delivered vaginally predicted hemostatic difficulty during the postpartum period. She was, therefore, treated with cryoprecipitate. In the woman who gave birth by cesarean section, although all factor VIII-related activities rose into the normal range, the additional surgical trauma anticipated in cesarean section prompted the use of cryoprecipitate as well.