Article
Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy.
Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation - 1 Sept 2017
Ardissino Gianluigi, Salardi Stefania, Berra Silvia, Colussi Giacomo, Cugno Massimo, Zecca Marco, Giglio Fabio, Peccatori Jacopo, Diral Elisa, Tel Francesca, Clivio Alberto, Tedeschi Silvana
Abstract excerpt
Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement...
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