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Hematopoietic stem cell transplantation‐associated thrombotic microangiopathy accompanied by renal arteriolar C4d deposition
Author(s) -
Sakamoto Kenichi,
Imamura Toshihiko,
Osone Shinya,
Nishimura Ayako,
Nishida Masashi,
Ishida Hiroyuki,
Hosoi Hajime
Publication year - 2015
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12450
Subject(s) - thrombotic microangiopathy , eculizumab , complement system , medicine , peritubular capillaries , complement membrane attack complex , alternative complement pathway , transplantation , atypical hemolytic uremic syndrome , pathology , immunology , antibody , disease
HSCT ‐associated thrombotic microangiopathy ( TA ‐ TMA ) is a severe complication with a poor prognosis. Recently, it has been reported that complement system dysregulation, such as CFH autoantibodies and deletions CFH ‐related genes 3 and 1, induced TA ‐ TMA . In addition, C4d‐positive renal arterioles are both a good marker of complement system activation and a useful diagnostic tool for TA ‐ TMA . Because dysregulation of the complement system is associated with TA ‐ TMA , the complement system might be a therapeutic target, such as eculizumab, a terminal complement inhibitor. Herein, we describe an eight‐yr‐old boy who developed TA ‐ TMA accompanied by severe renal dysfunction. His renal specimen showed diffuse C4d deposition in the renal arterioles, which is consistent with TA ‐ TMA . Although the patient gradually improved without eculizumab, renal arteriolar C4d staining in sample with TA ‐ TMA shows the complement system activation and may guide the target therapy using the eculizumab.