TY - JOUR
T1 - Hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation in children
T2 - A nationwide survey in Japan
AU - Asano, Takeshi
AU - Kogawa, Kazuhiro
AU - Morimoto, Akira
AU - Ishida, Yasushi
AU - Suzuki, Nobuhiro
AU - Ohga, Shouichi
AU - Kudo, Kazuko
AU - Ohta, Shigeru
AU - Wakiguchi, Hiroshi
AU - Tabuchi, Ken
AU - Kato, Shunichi
AU - Ishii, Eiichi
N1 - Copyright:
Copyright 2012 Elsevier B.V., All rights reserved.
PY - 2012/7/15
Y1 - 2012/7/15
N2 - Background: Hemophagocytic lymphohistiocytosis (HLH) is associated with hypercytokinemia in children. Although HLH can be also observed after hematopoietic stem cell transplantation (HSCT), the incidence and clinical features of HLH after HSCT remain obscure. Procedure: The clinical features of HLH after HSCT (post-HSCT HLH) were investigated in children with malignancies, immune deficiencies, or aplastic anemia. The HLH/Langerhans Cell Histiocytosis (LCH) Committee of the Japanese Society of Pediatric Hematology (JSPH) sent questionnaires to hospitals with JPSH members asking for details of cases in which HLH occurred after HSCT between 1998 and 2008. Results: Among 42 children who were diagnosed with post-HSCT HLH between 1998 and 2008 in Japan, 37 fulfilled our inclusion criteria; of these, 26 were classified as early-onset (onset <30 days after HSCT) and 11 were classified as late-onset (onset >30 days after HSCT). In the early-onset group, the presence of respiratory symptoms, high levels of total bilirubin, and triglycerides at onset and the lack of control of GVHD with tacrolimus were significantly associated with non-resolution of HLH (P<0.05). The survival rate was significantly higher in patients with resolution of HLH than in those without resolution (59% vs. 14%, P<0.05). Conclusions: These findings suggest that early-onset post-HSCT HLH is a specific entity of HLH, and appropriate diagnosis and prompt management need to be established.
AB - Background: Hemophagocytic lymphohistiocytosis (HLH) is associated with hypercytokinemia in children. Although HLH can be also observed after hematopoietic stem cell transplantation (HSCT), the incidence and clinical features of HLH after HSCT remain obscure. Procedure: The clinical features of HLH after HSCT (post-HSCT HLH) were investigated in children with malignancies, immune deficiencies, or aplastic anemia. The HLH/Langerhans Cell Histiocytosis (LCH) Committee of the Japanese Society of Pediatric Hematology (JSPH) sent questionnaires to hospitals with JPSH members asking for details of cases in which HLH occurred after HSCT between 1998 and 2008. Results: Among 42 children who were diagnosed with post-HSCT HLH between 1998 and 2008 in Japan, 37 fulfilled our inclusion criteria; of these, 26 were classified as early-onset (onset <30 days after HSCT) and 11 were classified as late-onset (onset >30 days after HSCT). In the early-onset group, the presence of respiratory symptoms, high levels of total bilirubin, and triglycerides at onset and the lack of control of GVHD with tacrolimus were significantly associated with non-resolution of HLH (P<0.05). The survival rate was significantly higher in patients with resolution of HLH than in those without resolution (59% vs. 14%, P<0.05). Conclusions: These findings suggest that early-onset post-HSCT HLH is a specific entity of HLH, and appropriate diagnosis and prompt management need to be established.
UR - http://www.scopus.com/inward/record.url?scp=84861205228&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84861205228&partnerID=8YFLogxK
U2 - 10.1002/pbc.23384
DO - 10.1002/pbc.23384
M3 - Article
C2 - 22038983
AN - SCOPUS:84861205228
SN - 1545-5009
VL - 59
SP - 110
EP - 114
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
IS - 1
ER -