Korean J Nephrol.  2002 Jul;21(4):610-617.

A Clinical Study on Adult Thrombotic Thrombocytopenic Purpura

• Park GH
• Lee HK
• Lee YM
• Choi DE
• Lee SJ
• Jo DY
• Lee KW
• Shin YT

Abstract

OBJECTIVE
Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological symptoms and a variable degree of impairment of renal function, which are thought to be due to endothelial cell injury, platelet activation and subsequent formation of thrombi in the microcirculaion. It usually occurs in adults but few reports are available on etiology, clinical manifestation, progression and the outcome of disease in Korea.
METHODS
We investigated 10 adult patients who had admitted and were diagnosed as TTP in Department of Internal Medicine, Chungnam National University Hospital from Dec. 1994 to Jul. 2001.
RESULTS
The male to female ratio was 1 : 4. The etiologic antecedants were infectious diarrhea in 3 patients, upper respiratory infection in 3 patients and pregnancy was related with TTP in 2 patents. The clinical manifestations were variable neurologic symptoms(100%), renal involvement(90%), hemorrhagic manifestations(80%), fever(60%), and diarrhea(40%). Acute renal failure was noted in 6 patients and hemodialysis was required in 5 patients. Plasma exchange was performed in 9 patients and corticosteroid was prescribed in 8 patients, simultaneously. Vincristine, azatioprine and cyclophosphamide were added in 2 patients. One patient died of hepatic failure. Seven patients showed complete recovery. One patient progressed to end-stage renal failure. The other patient showed multiple recurrences.
CONCLUSION
It is thought that a considerable number of TTP patients show very serious acute renal failure and plasma exchange should be included in therapeutic modalities of TTP.

Keyword

Thrombotic thrombocytopenic purpura; Thrombotic microangiopathy; Plasma exchange

MeSH Terms

Acute Kidney Injury
Adult*
Anemia, Hemolytic
Chungcheongnam-do
Cyclophosphamide
Diarrhea
Endothelial Cells
Female
Humans
Internal Medicine
Kidney Failure, Chronic
Korea
Liver Failure
Male
Plasma Exchange
Platelet Activation
Pregnancy
Purpura, Thrombotic Thrombocytopenic*
Recurrence
Renal Dialysis
Thrombocytopenia
Thrombotic Microangiopathies
Vincristine
Cyclophosphamide
Vincristine