Intravenous immunoglobulin as an adjunct to plasma exchange for the treatment of chronic thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. Therapeutic plasma exchange (TPE) is the most effective therapy; however, despite TPE, about one-third of TTP patients will relapse. A subset of patients with TTP has antibodies to ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) and may become resistant to conventional treatments. We describe a patient with TTP and high-titre anti-ADAMTS13 antibodies who developed a chronic, relapsing course of TTP despite frequent TPE. Once adjuvant treatment with intravenous immunoglobulin (IVIG) was added, remission was achieved. Even during remission, anti-ADAMTS13 antibodies remained elevated. We conclude that IVIG may sustain remission in some patients with chronic, relapsing TTP.
Department of Medicine, Michael G. De Groote School of Medicine, Health Sciences Centre, McMaster University, 1200 Main Street West, Hamilton, Ontario, Canada. moorej@mcmaster.ca
J C
JC
Moore
D M
DM
Arnold
B F
BF
Leber
R
R
Clare
G J
GJ
Molnar
J G
JG
Kelton
D051722:ADAM Proteins / Q000097:blood / Q000187:drug effects / Q000276:immunology*; D000071120:ADAMTS13 Protein; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D002908:Chronic Disease; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D010951:Plasma Exchange / Q000379:methods*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000628:therapy; D012074:Remission Induction / Q000379:methods; D055502:Secondary Prevention
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human