Analysis of clinical and laboratory characteristics in 42 patients with thrombotic thrombocytopenic purpura from a single center in China

作者：Deng, Ming-yang; Zhang, Guang-sen^*; Zhang, Yang; Xiao, Han; Dai, Chong-wen; Xu, Yun-xiao; Zheng, Wen-li; Peng, Hong-ling; Shen, Jian-kai

来源：Transfusion and Apheresis Science, 2013, 49(3): 447-452.

DOI：10.1016/j.transci.2013.07.026

• TTP
• Chinese
• Clinical feature
• ADAMTS 13
• T lymphocytic subset

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterized by microvascular platelet deposition and thrombus formation with resulting microangiopathic hemolytic anemia. Deficiency of the von Willebrand factor cleavage protease, also known as ADAMTS 13, has been implicated as an important etiological factor in TTP. Little studies were obtained on Chinese patients with UP until now. Our aim was to analyze the clinical features, outcome and laboratory characteristics of Chinese UP patients, and determine whether plasma ADAMTS 13 activity is decreased in UP and its diagnostic value for UP. Forty-two UP patients (29 females; 13 males) admitted to our hospital from 1998 to 2010 were analyzed. There were 34 patients (81%) with the triad of UP, including hemolytic anemia, thrombocytopenia and neurologic abnormalities; 7 (16.7%) had the classical pentad of UP. Major etiologic factors were acquired autoimmunological abnormalities (31%); no familial UP was identified in this series. The schistocytes of peripheral blood smears were present in all cases with a mean frequency of 4.6% (range from 0.3% to 13.4%). Plasma ADAMTS 13 activity was determined in 22 patients with the FRET-vWF86 assay. Only 4 idiopathic UP patients (18.2%) had severe ADAMTS 13 deficiency (activity < 10%); 9 (40.9%) had moderate decrease of ADAMTS 13 activity (activity: 10-40%); another 9 (40.91%) had normal ADAMTS 13 activity (>40%). T lymphocyte subpopulation was measured in 23 UP patients with FACS Calibur; 14 of the 23 (60.9%) had significantly decreased CD4 cells count and CD4/CD8 ratio, suggesting cellular immune dysfunction may be involved in the pathogenesis of UP. In the studies, plasmapheresis is the main therapeutic method. 26 of 31 patients (83.9%) accepting plasmapheresis achieved complete remission; those patients who only underwent plasma infusion had low remission rate (18.2%) and high mortality (9/11; 81.8%). Four patients with packed RBC infusion manifested transient exacerbation of neurologic or psychiatric symptoms. In conclusion, the diagnosis of UP in China is still based on clinical features including evidence of microangiopathic hemolysis. Severe ADAMTS 13 activity deficiency might be a valuable indicator for idiopathic TIT diagnosis. Further studies are needed to determine the real value of ADAMTS 13 activity for UP diagnosis and whether T lymphocytes subset dysregulation plays important role in UP pathogenesis.

• 出版日期2013-12
• 单位中南大学