Long-term treatment of refractory HIV-related immune thrombocytopenia in a patient with haemophilia A

Abstract

Haemophilia A patient developed symptomatic immune thrombocytopenia 5 years after HIV seroconversion without any progression of the viral disease. He displayed major bleeding with less than 30 x 10(9) platelets/l. No increase in platelet count was obtained using steroids, azidothymidine and alpha-interferon, while the patient was responsive only to high-dose intravenous immunoglobulins (IVGG). The patient remained responsive to IVGG for 1 year, and the repeated infusions of immunoglobulins were effective in safely maintaining the platelet count, with peak counts above 100 x 10(9)/l. On the contrary, after a single course of six plasma exchanges the patient became symptomatic and completely refractory to IVGG during the next month. In conclusion, IVGG could be effectively used in a long-term regimen in haemophiliacs with refractory HIV-ITP to avoid the risk of haemorrhages and to delay splenectomy.