Thrombocytopenic Purpura Essay

Part 1: Overview current literature about Thrombotic Thrombocytopenic Purpura

The first patient described with thrombotic thrombocytopenic purpura (TTP) is a 16 year old girl that died within 2 weeks after disease onset. She struggled with petechial bleeding, pallor, fever, paralysis, hematuria and coma (Lämmle et al. 2005). From the autopsy in 1924 Doctor E. Moschcowitz described hyaline thrombosis of the terminal arterioles and capillaries and he suspected that a powerful agglutinative and hemolitic poison was responsible for the disease (Moschcowitz 1924; Moschcowiz 1925). Four decades after the first diagnosis, five general diagnostic criteria were established. The clinical and laboratory features are fever, haemolytic anemia, purpura or other bleeding, transient or permanent neurologic signs and renal disease (Amorosi and Ultmann 1966) and are still used as the diagnostic hallmarks.

Moake et al. detected the presence of unusually large von Willebrand factor (ULVWF) multimers in plasma of patients with TTP and suspected that the ULVWF mulitmers were responsible for platelet clumping in the microvasculature. Moreover, they hypothesised that there was a lack of a depolymerase for the ULVWF (Moake et al. 1982). From then on, the lack of ULVWF depolymerase or the VWF-cleaving protease (VWFcp) was known to be responsible for TTP and was further established from then on (Lämmle et al. 2005; Furlan 2004; Tsai 2004; Moake