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

Research output: Contribution to journalArticle

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.
Original languageEnglish
Pages (from-to)1580-1582
Number of pages3
JournalBiology of Blood and Marrow Transplantation
Volume23
Issue number9
DOIs
Publication statusPublished - Sep 1 2017

Keywords

  • Complement
  • Complement gene mutations
  • Hematopoietic stem cell transplantation (HSCT)
  • Hemolytic uremic syndrome
  • Thrombotic microangiopathy (TMA)

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