S. Nagaya et al., HEMOSTATIC ABNORMALITIES AND INCREASED VASCULAR ENDOTHELIAL-CELL MARKERS IN PATIENTS WITH RED-CELL FRAGMENTATION SYNDROME INDUCED BY MITOMYCIN-C, American journal of hematology, 50(4), 1995, pp. 237-243
We examined red cell fragmentation syndrome (RCFS) induced by mitomyci
n C (MMC) (13 patients), by thrombotic thrombocytopenic purpura (TTP)
(17 patients), and by disseminated intravascular coagulation (DIC) (15
patients), Plasma cytokine levels were increased in the TTP and DIC p
atients, but not in those whose RCFS was induced by MMC, suggesting th
at the activation of the immune system plays an important role in the
pathogenesis of RCFS due to TTP and DIC but did not in RCFS due to MMC
, Plasma thrombomodulin, tissue type plasminogen activator, and plasmi
nogen activator inhibitor-I levels were increased in all RCFS patients
, suggesting that RCFS, whether MMC induced, or due to TTP or DIG, mig
ht be associated with vascular endothelial cell injury, In TTP, von Wi
llebrand factor (vWF) antigen and high molecular weight vWF multimer l
evels were reduced, possibly as a result of microthrombus consumption,
The hemostatic data in this study showed that the TTP patients were i
n a hypercoagulable state without hyperfibrinolysis, and that DIC pati
ents were in both a hypercoagulable and a hyperfibrinolytic state, whe
reas hemostatic abnormalities were slight in patients with MMC induced
RCFS, These findings suggest that vascular endothelial cell injuries
might be associated with RCFS, and that those injuries in MMC-induced
RCFS might not be related to microthrombi or an activated immune syste
m. (C) 1995 Wiley-Liss, Inc.