title: [Allogeneic hematopoietic stem cell transplantation associated thrombotic microangiopathy: 16 cases report and literature review].
abstract: OBJECTIVE
  To analyze the clinical characteristics, treatment and prognosis of 16 allogeneic hematopoietic stem cell transplantation (allo- HSCT)- associated thrombotic microangiopathy (TA- TMA) patients.
  
  
  METHODS
  The clinical data of 16 TA- TMA cases in 852 patients following allo- HSCT from Jan. 2013 to Jun. 2015 in the First Affiliated Hospital of Soochow University were retrospectively analyzed.
  
  
  RESULTS
  Of all the 852 allo-HSCT recipients, 16 patients were diagnosed as TA-TMA and the 1-year cumulative incidence of TA-TMA was (2.3±0.6)%. Among them, there were 9 males and 7 females, the median age was 41-year-old (12-54), and the median times of diagnosis of TA-TMA were 72 (21- 525) days after HSCT. Additionally, the median platelet counts, hemoglobin, percentage of schistocytes and Lactate dehydrogenase (LDH) levels were 20(11-36) ×10(9)/L, 74(56-99) g/L, 3% (2%- 13%) and 762(309-1 049) U/L, respectively. All 16 cases have normal ADAMTS13 level (over 60%), 10 patients had neurologic dysfunction and elevated creatinine were seen in 7. The major treatment of TA-TMA was withdrawn of calcineurin inhibitors, plasma exchange and corticosteroids. Finally, 8 patients achieved response after treatment and the other patients died of poor response. Compared with TA- TMA who achieved remission after therapy, those who got no response after interventions presented acute GVHD and they had higher schistocytes (62.5% cases>5% vs all cases ≤4%), LDH [826 (674-1 310) U/L vs 636 (309- 941) U/L] and serum creatinine levels [127 (70- 215) μmol/L vs 56 (22- 101) μmol/L].
  
  
  CONCLUSIONS
  TA-TMA was a severe complication after allo-HSCT, it could progress to multi-organ injury and was associated with poor outcome, the therapeutic efficacy depends on disease severity and coexisted complications.
authors: The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.
authors: W
authors: W
authors: Han
authors: Y
authors: Y
authors: Han
authors: J
authors: J
authors: Chen
authors: X
authors: X
authors: Ma
authors: F
authors: F
authors: Chen
authors: X J
authors: XJ
authors: Wu
authors: J Q
authors: JQ
authors: Qi
authors: H Y
authors: HY
authors: Qiu
authors: A N
authors: AN
authors: Sun
authors: D P
authors: DP
authors: Wu
mesh_terms: D000293:Adolescent; D000328:Adult; D065095:Calcineurin Inhibitors; D002648:Child; D005260:Female; D006086:Graft vs Host Disease; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D010976:Platelet Count; D011379:Prognosis; D012189:Retrospective Studies; D057049:Thrombotic Microangiopathies / Q000209:etiology* / Q000628:therapy*; D055815:Young Adult
chemical_list: D065095:Calcineurin Inhibitors