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Transient familial haemophagocytic lymphohistiocytosis reactivation post‐CD34 haematopoietic stem cell transplantation
Author(s) -
Almousa H.,
OuachéeChardin M.,
Picard C.,
RadfordWeiss I.,
CaillatZucman S.,
CavazzanaCalvo M.,
Blanche S.,
Saint Basile G.,
Le Deist F.,
Fischer A.
Publication year - 2005
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2005.05615.x
Subject(s) - immunology , stem cell , haematopoiesis , medicine , transplantation , hematopoietic stem cell transplantation , hemophagocytic lymphohistiocytosis , bone marrow , macrophage activation syndrome , perforin , immune system , biology , pathology , disease , cd8 , genetics , arthritis
Summary Familial haemophagocytic lymphohistiocytosis (FHLH) is a genetic disorder caused by defective lymphocyte cytotoxicity, resulting in impaired lymphocyte homeostasis and macrophage infiltration of solid tissues and bone marrow, with extensive haemophagocytosis. It is invariably fatal unless treated by allogeneic haematopoietic stem cell transplantation (HSCT). In a retrospective analysis of 11 cases of FHLH, transplanted in one centre between January 1999 and December 2003, it was found that host T cell expansion occurred early after HSCT in a setting of a viral infection (cytomegalovirus and Epstein–Barr virus respectively) in two cases who received T cell‐depleted HSCT. Transient recurrence of clinical and biological manifestations of FHLH was observed, despite evidence for donor cell engraftment. Secondary development of donor T cells led to stable mixed chimaerism and sustained remission of FHLH. Detection of host‐derived T cells soon after HSCT in a patient with FHLH should thus not mistakenly be taken as a manifestation of graft rejection.