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In Reply: Drs El-Rahman and Ammar suggest an equivalence between TMA and TTP, and advocate for plasma exchange with fresh-frozen plasma. Thrombotic microangiopathy represents a syndrome that may result from different conditions, which include TTP, hemolytic-uremic syndrome, acute postpartum and contraceptive-associated renal failure, malignant hypertension, HELLP (hemolysis, elevated liver enzymes, and low platelet count in association with eclampsia) syndrome, cancer, immunosuppressive therapy, and catastrophic antiphospholipid syndrome.^1-2 Not all share the severe deficiency of, or the inhibitory activity against, the von Willebrand factor–cleaving protease ADAMTS-13, which is present in TTP, leaving unclear the role of plasma exchange in the treatment of (for example) adult patients with hemolytic-uremic syndrome.³

In some cases, TMA is probably related to the presence of an antibody, similar to catastrophic antiphospholipid syndrome. In an experimental model of TMA mimicking TTP, removal of the plasma was sufficient to establish remission without needing infusion of healthy plasma.⁴ A review . . . [Full Text of this Article]

Javier Fernández, MD
jfdez@clinic.ub.es

Francesc Balaguer, MD
Liver Unit
Institut de Malalties Digestives

Miguel Lozano, MD
Hemotherapy and Hemostasis Department
Hospital Clinic Barcelona
University of Barcelona
Barcelona, Spain

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