Decreased activity of plasma ADAMTS-13 predicts poor prognosis in acute lymphoblastic leukemia patients after hematopoietic stem cell transplant

• Published in: Journal of hematopathology Vol. 13; no. 1; pp. 41 - 46
• Main Authors: Li, Zhenxue, Chen, Xiaoning, Lu, Songsong, Zhao, Lei, Zhu, Mengjie, Xu, Qinzhu, Song, Ying, Liu, Chen
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.03.2020
• Subjects: Hematology; Medicine; Medicine & Public Health; Original Article; Pathology; ADAMTS-13; Prognosis; Acute lymphoblastic leukemia; HSCT
• ISSN: 1868-9256; 1865-5785
• DOI: 10.1007/s12308-019-00382-8

Decreased ADAMTS-13 is important for the diagnosis of thrombotic thrombocytopenic purpura (TTP), but recent studies have shown that reduced ADAMTS-13 activity is associated with disease prognosis of a variety of cancer patients. As an important hematological malignancy, the significance of ADAMTS-13 in acute lymphoblastic leukemia (ALL) has not been fully elucidated. This research aims to explore the significance of decreased ADAMTS-13 activity in acute lymphoblastic leukemia after hematopoietic stem cell transplant (HSCT). Thirty-eight ALL patients were included in this research, and their ADAMTS-13 activity was measured before HSCT, including 21 patients with low ADAMTS-13 activity (< 481 ng/ml) and 17 patients with normal ADAMTS-13 activity (481–785 ng/ml). Related medical indicators before HSCT and 1 month after HSCT were collected. All the patients were followed and their progress was recorded and evaluated. Level changes of related medical indicators and prognosis situations were compared between two groups. ALL patients with low ADAMTS-13 activity suffered more HSCT-related complications than patients with normal ADAMTS-13 activity. There was no significant difference for APTT, PT, CRP, and D-dimer between two groups. Low ADAMTS-13 Group underwent higher mortality rate than Normal Group during the 1-year follow-up after HSCT and 2-year follow-up after the onset of ALL. To conclude, low ADAMTS-13 activity predicts poor prognosis of ALL after HSCT.