Two Cases of Systemic Lupus Erythromatosis with Manifestation of Thombotic Thrombocytopenic Purpura.
- Author:
Young Ki LEE
1
;
Young Joo KWON
;
Gu LEE
;
Jong Woo YOON
;
Sang Kyung JO
;
Dae Ryong CHA
;
Won Yong CHO
;
Heui Jung PYO
;
Hyoung Kyu KIM
;
Nam Hee WOEN
Author Information
1. Department of Internal Medicine and Pathology, College of Medicine, Korea University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Thrombotic thrombocytopenic purpura;
Systemic lupus erythromatosus
- MeSH:
Adult;
Anemia, Hemolytic;
Biopsy;
Cyclophosphamide;
Diagnosis;
Dyspnea;
Early Diagnosis;
Female;
Fever;
Humans;
Nausea;
Plasma;
Plasmapheresis;
Purpura, Thrombocytopenic*;
Purpura, Thrombotic Thrombocytopenic;
Recurrence;
Thrombocytopenia;
Thrombotic Microangiopathies;
Vomiting;
Young Adult
- From:Korean Journal of Nephrology
1997;16(3):584-590
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Thrombotic thrombocytopenic purpura(TTP) is a clinical syndrome of unknown etiology and characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological status, renal dysfunction and fever. Systemic lupus erythromatosus(SLE) is also multisystemic disease that some of clinical features may mimic TTP. Therefore both diseases have led to diagnostic confusion. We experienced two cases with SLE who subsequently or initially developed TTP. In case 1, a 44-year old woman had 1-year previous history of SLE and presented with dyspnea. After diagnosis of thrombotic microangiopathy by renal biopsy, she was managed with steroid, cyclophosphamide pulse therapy, fresh frozen plasma infusion and plasmapheresis. She was treated by aggressive treatment; nevertheless, she died on 15th admission day. In case 2, a 22-year old man was admitted because of nausea and vomiting. SLE with TTP was diagnosed by ARA criteria and the finding of microangiopathic hemolytic anemia. He was treated with plasmapheresis, fresh frozen plasma infusion and steroid therapy. He showed clinical response to the therapy, and has shown no recurrence of disease until now on. In conclusion, we suggest that early diagnosis and prompt therapy such as plasmapheresis and plasma infusion are very important in SLE with TTP.
