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Nephron Clin Pract. 2009;112(3):c177-83. doi: 10.1159/000218106. Epub 2009 May 13.

Clinicopathological characteristics and outcome of Chinese patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: a 9-year retrospective study.

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1
Department of Nephrology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, PR China.

Abstract

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.

METHOD:

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.

PMID:
19439988
DOI:
10.1159/000218106
[Indexed for MEDLINE]
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