Korean J Nephrol.  1997 Sep;16(3):584-590.

Two Cases of Systemic Lupus Erythromatosis with Manifestation of Thombotic Thrombocytopenic Purpura

• Lee YK
• Kwon YJ
• Lee G
• Yoon JW
• Jo SK
• Cha DR
• Cho WY
• Pyo HJ
• Kim HK
• Woen NH

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.

Keyword

Thrombotic thrombocytopenic purpura; Systemic lupus erythromatosus

MeSH Terms

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
Cyclophosphamide