Clinicopathological characteristics and outcome of Chinese patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: a 9-year retrospective study.
BACKGROUND
The pathogenesis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is unclear and the prognosis is poor. Few studies have been published focusing on Chinese patients with TTP-HUS. We performed a retrospective study on the clinical characteristics and outcome of Chinese patients with TTP-HUS.


METHODS
Patients with TTP-HUS, admitted to our hospital from 1998 to 2006, were retrospectively analyzed.


RESULTS
There were 26 females and 6 males in our study. Fifteen patients had systemic lupus erythematosus (SLE)-associated TTP-HUS; 2 had pregnancy-associated TTP-HUS; 1 had antiphospholipid syndrome-associated TTP-HUS; 2 had drug-associated TTP-HUS; 4 had malignant angionephrosclerosis- associated TTP-HUS; 3 had vasculitis-associated TTP-HUS, and the remaining 5 had idiopathic TTP-HUS. Twenty-six patients had acute kidney injury and 21 had nephrotic syndrome. Hypertension was found in 31 patients. For the treatment, 15 patients had plasmapheresis, 12 had continuous veno-venous hemodiafiltration and 14 had hemodialysis. Eighteen patients were treated with intravenous immunoglobulin. Corticosteroids were used in patients with idiopathic TTP-HUS. For the patients with SLE-associated TTP-HUS, corticosteroids and immunosuppressant were used. Outcome was poor: 6 patients died; 17 recovered from renal insufficiency; 5 progressed to chronic renal failure, and 4 were dependent on hemodialysis.


CONCLUSIONS
Most of our patients had secondary TTP-HUS. SLE-associated TTP-HUS is the most common form of TTP-HUS. Early diagnosis and treatment can improve prognosis. An immunosuppressant together with corticosteroids could improve prognosis in some patients.
Zhang
Wen
W
Department of Nephrology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, PR China.
Shi
Hao
H
Ren
Hong
H
Shen
Ping-Yan
PY
Pan
Xiao-Xia
XX
Li
Xiao
X
Chen
Yong-Xi
YX
Xu
Yao-Wen
YW
Chen
Xiao-Nong
XN
Zhu
Ping
P
Chen
Nan
N
D000293:Adolescent; D000328:Adult; D002648:Child; D002681:China / Q000453:epidemiology; D015897:Comorbidity; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy* / Q000401:mortality*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000401:mortality*; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
D007166:Immunosuppressive Agents