[Thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus successfully treated with plasma exchange and corticosteroids].
Thrombotic thrombocytopenic purpura (TTP) is a rare dynamic life-threatening disease with systemic formation of platelet thrombi in the microvasculature within all the organs. Until recently the mortality in TTP has exceeded 90%. The progress of medical research in the last two decades has significantly improved our understanding of the pathogenesis of TTP and allowed a reduction in mortality. This paper describes a case of a 36-year-old female patient with systemic lupus erythematosus (SLE) who was admitted to our hospital for jaundice and speech disturbances. Laboratory tests revealed hemolytic anemia with a negative Coombs test. Abundant schistocytes were present in peripheral blood smear. During the first day of hospitalization progression of neurological signs was observed -the patient received high-dose corticosteroids (1000 mg of methylprednisolone daily for 5 consecutive days) and underwent plasma exchange therapy. We observed significant improvement of the patient's condition, as well as a complete resolution of clinical and laboratory abnormalities. In the following 24 months there have been no signs of the relapse of TTP. The article contains also a brief update of this unusual disease.
Wiśniewski
Piotr
P
Klinika Endokrynologii i Chorób Wewnetrznych, Akademia Medyczna, Gdańsk. piotr.wisniewski@amg.gda.pl
Szarejko-Kaska
Monika
M
Sworczak
Krzysztof
K
Hellmann
Andrzej
A
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000328:Adult; D003298:Coombs Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008775:Methylprednisolone / Q000008:administration & dosage; D010951:Plasma Exchange / Q000379:methods*; D010956:Plasmapheresis; D020551:Pulse Therapy, Drug; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D012074:Remission Induction
D000305:Adrenal Cortex Hormones; D008775:Methylprednisolone