RESEARCH - A comparison of TTP in an inception cohort of patients with and without SLE

[Guest guest]

Rheumatology Advance Access published online on February 6, 2009

Rheumatology, doi:10.1093/rheumatology/ken510

A comparison of thrombotic thrombocytopenic purpura in an inception

cohort of patients with and without systemic lupus erythematosus

Pagalavan Letchumanan1, Heng-Joo Ng2, Lai-Heng Lee2 and n Thumboo3,4

1School of Medicine and Health Sciences, Monash University (Sunway

Campus), Johor Bahru, Malaysia, 2Department of Haematology,

3Department of Rheumatology and Immunology, Singapore General Hospital

and 4Department of Medicine, Yong Loo Lin School of Medicine, National

University of Singapore, Singapore

Abstract

Objectives. To compare the clinical presentation, response to therapy

and outcome of thrombotic thrombocytopenic purpura (TTP) in an

inception cohort of patients with and without SLE.

Methods. Medical records of patients diagnosed with TTP at Singapore

General Hospital between January 2003 and December 2007 were reviewed.

Results. Ten idiopathic TTP (iTTP) and eight SLE-associated TTP (sTTP)

patients were identified, with iTTP patients being older (mean 50.4 vs

34.5 yrs). Five iTTP patients were ANA positive but did not have any

features of SLE. All sTTP patients had active SLE at TTP diagnosis and

had more renal involvement than iTTP (87.5% vs 50%). The mean duration

from the first symptom suggestive of TTP to diagnosis was 7.7 days and

19.5 days in iTTP and sTTP patients. All patients received high-dose

corticosteroids. Cytotoxic and immunosuppressive drugs were used more

commonly (87.5% vs 50%) and earlier (Day 2/3 vs after Day 7) in sTTP

patients. Vincristine was the drug of choice in iTTP and

cyclophosphamide in sTTP. Three SLE patients received rituximab.

Mortality for iTTP and sTTP was 50% (95% CI 19%, 81%) and 62.5% (95%

CI 29%, 96%), respectively. The mean (S.D.) time to complete remission

was 31.3 (± 26.4) days in sTTP (n = 3) and 16.8 (± 6.1) days in iTTP

(n = 5).

Conclusion. Despite early and more aggressive therapy in sTTP,

mortality was higher and the time to complete remission were longer,

suggesting that sTTP is more severe. The tempo of development of TTP

in SLE patients was slower.

http://rheumatology.oxfordjournals.org/cgi/content/abstract/ken510v1?papetoc

Not an MD