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.
Department of Nephrology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, PR China.
Wen
W
Zhang
Hao
H
Shi
Hong
H
Ren
Ping-Yan
PY
Shen
Xiao-Xia
XX
Pan
Xiao
X
Li
Yong-Xi
YX
Chen
Yao-Wen
YW
Xu
Xiao-Nong
XN
Chen
Ping
P
Zhu
Nan
N
Chen
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