Transplantation-associated thrombotic microangiopathy: twenty-two years later.
A syndrome of microangiopathic hemolytic anemia, renal dysfunction and neurological abnormalities was first noted in bone marrow transplant recipients 22 years ago. Now known as transplantation-associated thrombotic microangiopathy (TA-TMA) to distinguish it from other thrombotic microangiopathies, this disorder responds poorly to conventional treatments for thrombotic thrombocytopenic purpura. In this review, we discuss the incidence and risk factors for TA-TMA and describe a pathophysiologic model of the disorder based on results obtained from laboratory models of the thrombotic microangiopathies. We conclude by suggesting possible approaches to the early diagnosis and treatment of TA-TMA based on this model that may warrant testing in future clinical trials.
Daly
A S
AS
Department of Medical Oncology and Hematology, Princess Margaret Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada.
Xenocostas
A
A
Lipton
J H
JH
D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D004195:Disease Models, Animal; D004730:Endothelium, Vascular / Q000473:pathology; D006801:Humans; D008833:Microcirculation / Q000473:pathology; D011697:Purpura, Thrombotic Thrombocytopenic; D013921:Thrombocytopenia / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy