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 language | English |
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Pages (from-to) | 1580-1582 |
Number of pages | 3 |
Journal | Biology of Blood and Marrow Transplantation |
Volume | 23 |
Issue number | 9 |
DOIs | |
Publication status | Published - Sep 1 2017 |
Keywords
- Complement
- Complement gene mutations
- Hematopoietic stem cell transplantation (HSCT)
- Hemolytic uremic syndrome
- Thrombotic microangiopathy (TMA)