Acquired thrombotic thrombocytopenic purpura as the presenting symptom of systemic lupus erythematosus. Successful treatment with plasma exchange and immunosuppression--report of two cases.
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening syndrome characterized by platelet aggregation causing occlusive microangiopathy. It has been described as a complication in systemic lupus erythematosus (SLE). Recent research indicated that genetic or autoantibody-induced deficiency of the metalloprotease ADAMTS13 plays a key role in the pathogenesis of TTP. Here we report two uncommon cases of TTP as the first presenting symptom of SLE. Both patients were treated with combined plasma exchange and immunosuppressive therapy, and recovered completely. Although TTP and SLE have several clinical findings in common, and both disorders may coexist more frequently than we currently assume, features of one disease should not mislead to reject the alternative disorder.
Starck
M
M
Department of Hematology, Städtisches Klinikum München GmbH, Krankenhaus München Schwabing, Munich, Germany. m.starck@extern.lrz-muenchen.de
Abedinpour
F
F
Dendorfer
U
U
Wagner-Czekalla
J
J
Pachmann
M
M
Mann
J F
JF
Nerl
C
C
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000328:Adult; D003298:Coombs Test; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008666:Metalloendopeptidases / Q000378:metabolism; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D016896:Treatment Outcome
D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human