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.
Moore
J C
JC
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
Arnold
D M
DM
Leber
B F
BF
Clare
R
R
Molnar
G J
GJ
Kelton
J G
JG
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