Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy
Articolo
Data di Pubblicazione:
2017
Citazione:
Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy / G. Ardissino, S. Salardi, S. Berra, G. Colussi, M. Cugno, M. Zecca, F. Giglio, J. Peccatori, E. Diral, F. Tel, A. Clivio, S. Tedeschi. - In: BIOLOGY OF BLOOD AND MARROW TRANSPLANTATION. - ISSN 1083-8791. - 23:9(2017 Oct), pp. 1580-1596. [10.1016/j.bbmt.2017.05.013]
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.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Complement; Complement gene mutations; Hematopoietic stem cell transplantation (HSCT); Hemolytic uremic syndrome; Thrombotic microangiopathy (TMA)
Elenco autori:
G. Ardissino, S. Salardi, S. Berra, G. Colussi, M. Cugno, M. Zecca, F. Giglio, J. Peccatori, E. Diral, F. Tel, A. Clivio, S. Tedeschi
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