Pathogenesis and treatment of disseminated thrombotic microangiopathy (Moschcowitz’ syndrome)
Issue title: Microcirculation, Interstitium, Lymph, Pathophysiology and Disease. Proceedings of the International Symposium, Villa La Principessa, Lucca, Italy, June 19–20, 1981
Affiliations: Division of Hematology, Dept. Int. Med. University Hospital, 10 Geert Grooteplein, 6525 GA Nijmegen, The Netherlands
Abstract: Disseminated thrombotic microangiopathy was until recently a fatal disease consisting of thrombocytopenic purpura, hemolytic anemia and progressive focal neurologic disturbances. Pathologically widespread hyaline occlusions are found in terminal arterioles and capillaries, which show enthothelial hyperplasia. The thrombi consist of platelets. This syndrome was first described by Moschcowitz in 1925 and is known in literature as thrombotic thrombocytopenic purpura (T.T.P.). In 1977 Byrnes and Khurana reported dramatic therapeutic responses in this hitherto fatal disorder, obtained by plasma infusions. Nine patients are described of which six were treated by massive plasma infusions, four which recovered completely. The experiences suggest the existence of a plasma factor that is lacking or consumed in these patients and the producticn of which is modulated by the levels of estrogen/progestagen in the plasma. Identification of this anti-T.T.P. factor will be of great interest for understanding and treatment of T.T.P. and of increased platelet aggregation tendencies in general.
