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
Gianluigi ArdissinoSilvana Tedeschi

Abstract

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 dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.

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Citations

May 7, 2021·Nature Reviews. Nephrology·Fadi Fakhouri, Véronique Frémeaux-Bacchi
Aug 2, 2017·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Eleni GavriilakiAchilles Anagnostopoulos
Dec 30, 2021·Thrombosis and Haemostasis·Evangeline Millicent RodriguesLuigi Porcaro

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