Difficulties in the management of an incomplete form of refractory thrombotic thrombocytopenic purpura, the usefulness of vincristine

Background: Although several etiologies can be identified in thrombotic thrombocytopenic purpura (TTP), idiopathic cases are still frequent. Incomplete forms are more rare. Currently, the diagnosis may be made in cases of thrombocytopenia and microangiopathic hemolytic anemia. According to the literature, mortality and morbidity are significantly improved with plasma exchange. However, treatment in refractory forms remains problematic.

Case Report: A 33-year old woman presented with an incomplete form of TTP, refractory to a combination of therapeutics. The patient underwent plasma infusion, plasma exchange, and then was started on corticosteroids. She also received intravenous immunoglobulins and antiplatelet agents in close proximity to vincristine (Oncovin®) infusion. The main biological indicators used were the platelet count, hematocrit, LDH, and the presence of schistocytes. Following vincristine treatment, the patient’s condition rapidly improved.

Conclusion: Vincristine administered after the failure of standard therapeutics was effective in this refractory form of TTP.