[Moschcowitz's disease--effect of therapeutic plasma exchange and splenectomy]

Moszkowicz's disease (thrombotic thrombocytopenic purpura), TTP, is an uncommon haematologic, neurologic syndrome which, almost regularly, terminates fatally. The disorder has a turbulent and fulminant course and is of short duration. The presented patient was treated with a combined therapeutic modality of intensive plasma exchange and subsequent splenectomy. This is the first case of successful treatment of TTP in Yugoslavia. The previous therapeutic approaches with infusions of fresh frozen plasma produced only transient or dubious responses. Clinical remissions were rare. In this patient, however, the plasma exchange with volume adjustment with the fresh frozen plasma or albumin produced restitution of the thrombocyte count to normal values and disappearance of all neurological signs and complaints. At that point surgical splenectomy was carried out with the concomitant extirpation of an accessory spleen circumstantially detected on exploration. Pathohistology of both spleens revealed typical tissue changes (disseminated hyaline and thrombocyte thrombi of small blood vessels) characteristic of Moszkowicz's disease. Following an uneventful splenectomy and discontinuation of all medicamentouse therapy the normal thrombocyte count was maintained without no sign of recurrence of neurological symptoms or manifestations.