Thrombotic thrombocytopenic purpura-like thrombocytopenia closely related to uremia: report of two cases.
Idiopathic thrombotic thrombocytopenic purpura (TTP) was suspected in two cases with end-stage renal disease (ESRD) before initiating dialysis based on both the clinical course and the initial laboratory findings. After three plasma exchanges and four sessions of hemodialysis, the thrombocytopenia improved to the normal range. However, the level of haptoglobin remained low after the recovery of platelet count. The low activity of ADAMTS13 (a disintegrin-like and metalloprotease with thrombospodin type 1 repeats) was not observed and the inhibitor was negative in these two cases. Therefore, thrombocytopenia was not attributable to TTP and other factors might therefore be involved in its pathogenesis. Some investigators have reported that a uremic state may possibly be involved in thrombocytopenia in both animals and humans. In the present cases, no apparent cause other than uremia was found because, after initiating hemodialysis, the thrombocytopenia rapidly improved. In conclusion, TTP-like thrombocytopenia can be caused by uremia and the influence of uremia should be considered in the pathogenesis of ESRD patients with TTP-like manifestations.
Nakamura
Hironori
H
Department of Nephrology, Showa University School of Medicine, Tokyo, Japan. hironori-nakamura@db3.so-net.ne.jp
Mukai
Masanori
M
Honda
Hirokazu
H
Akizawa
Tadao
T
D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology*; D014511:Uremia / Q000150:complications*
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human