[The dynamic analysis and the clinical significance of vascular endothelial cell markers and hemolysis parameters in thrombotic thrombocytopenic purpura].
OBJECTIVE
To monitor the changes of hemolysis parameters and endothelial cell markers in thrombotic thrombocytopenic purpura (TTP) and reveal the clinical significance of these changes.


METHODS
vWF-cleaving protease (vWF-CP) activity in 3 cases of TTP was detected by Western blot. The percentages of fragmented red cells (FRC) were counted throughout the entire clinical course. Levels of plasma thrombomodulin were detected by Western blot combined with density screening in TTP and healthy individuals (n = 3). Concentration of plasma VEGF was measured by enzyme-linked immunosorbent assay in TTP and healthy individuals (n = 9). Fundus fluorescein angiography was performed to search the evidence of microvascular thrombosis in one TTP patient with impaired visual acuity.


RESULTS
The lower vWF-CP activity was observed in TTP patients; the percentages of FRC in 3 cases of TTP were 1.65%, 2.50%, 3.32% respectively with an average of 2.49% at the onset of and decreased with the improvement of the disease. The levels of plasma TM and VEGF were significantly elevated in TTP than those in healthy individuals, and related to the severity of TTP. Fundus photography in one TTP patient with impaired visual acuity revealed vascular occlusion in fundus arteriole and venulae.


CONCLUSIONS
A decreased vWF-CP activity is in favour of TTP diagnosis. Dynamic monitoring of plasma TM and VEGF as well as percentages of FRC are useful indexes for reflecting the severity and evaluating therapeutic response of TTP. Selective fundus fluorescein angiography is useful for the judgement of microvascular thrombosis in TTP.
Deng
Ming-yang
MY
Division of Hematology, The Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhang
Guang-sen
GS
Li
Bin
B
Dai
Chong-wen
CW
Pei
Min-fei
MF
D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D015415:Biomarkers / Q000097:blood; D015153:Blotting, Western; D042783:Endothelial Cells / Q000378:metabolism; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006461:Hemolysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000473:pathology; D018180:Thrombomodulin / Q000097:blood*; D013997:Time Factors; D042461:Vascular Endothelial Growth Factor A / Q000097:blood*; D055815:Young Adult
D015415:Biomarkers; D018180:Thrombomodulin; D042461:Vascular Endothelial Growth Factor A; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human