TY - JOUR
T1 - Thrombotic Thrombocytopenic Purpura Syndrome in Systemic Lupus Erythematosus
T2 - Treatment With Plasma Infusion
AU - Gelfand, Janice
AU - Truong, Luan
AU - Stern, Leonard
AU - Pirani, Conrad L.
AU - Appel, Gerald B.
N1 - Funding Information:
From the Division of Nephrology. Department of Medicine. and the Department of Pathology. College of Physicians and Surgeons of Columbia University. New York. Supported in part by NIH Grants No. RR-00645-/3 and RO IOAM-25624. Address reprint requests to Gerald B. Appel. Department of Medicine. Room 454. Presbyterian Hospital. 622 W 168th St. New York. NY 10032. © 1985 by the National Kidney Foundation. Inc. 0272-6386/85/0603-()()()4$03.00/0
PY - 1985
Y1 - 1985
N2 - Two patients with well documented systemic lupus erythematosus developed a syndrome resembling thrombotic thrombocytopenic purpura. Both had severe thrombocytopenia, microangiopathic hemolytic anemia, seizures, and renal dysfunction. Prothrombin time, partial thromboplastin time, thrombin time, and fibrinogen levels were normal; fibrin degradation products were minimally elevated. Histologic evaluation of renal biopsies in both patients confirmed the impression of intravascular thrombosis. Therapy with corticosteroids, other immunosuppressive drugs and splenectomy (in one case) proved unsuccessful. The infusion of fresh frozen plasma, with or without plasmapheresis, reversed the syndrome. This report indicates that patients with systemic lupus may develop a thrombotic thrombocytopenic purpura like syndrome which responds to fresh plasma infusion.
AB - Two patients with well documented systemic lupus erythematosus developed a syndrome resembling thrombotic thrombocytopenic purpura. Both had severe thrombocytopenia, microangiopathic hemolytic anemia, seizures, and renal dysfunction. Prothrombin time, partial thromboplastin time, thrombin time, and fibrinogen levels were normal; fibrin degradation products were minimally elevated. Histologic evaluation of renal biopsies in both patients confirmed the impression of intravascular thrombosis. Therapy with corticosteroids, other immunosuppressive drugs and splenectomy (in one case) proved unsuccessful. The infusion of fresh frozen plasma, with or without plasmapheresis, reversed the syndrome. This report indicates that patients with systemic lupus may develop a thrombotic thrombocytopenic purpura like syndrome which responds to fresh plasma infusion.
KW - Thrombotic thrombocytopenic purpura
KW - plasma infusion
KW - systemic lupus erythematosus
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U2 - 10.1016/S0272-6386(85)80019-6
DO - 10.1016/S0272-6386(85)80019-6
M3 - Article
C2 - 4041134
AN - SCOPUS:0022415574
SN - 0272-6386
VL - 6
SP - 154
EP - 160
JO - American Journal of Kidney Diseases
JF - American Journal of Kidney Diseases
IS - 3
ER -