Hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation in children: a nationwide survey in Japan.
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.


METHODS
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.
Department of Pediatrics, Nippon Medical School, Chiba Hokusoh Hospital, Inzai City, Chiba, Japan. vff13540@nifty.ne.jp
Takeshi
T
Asano
Kazuhiro
K
Kogawa
Akira
A
Morimoto
Yasushi
Y
Ishida
Nobuhiro
N
Suzuki
Shouichi
S
Ohga
Kazuko
K
Kudo
Shigeru
S
Ohta
Hiroshi
H
Wakiguchi
Ken
K
Tabuchi
Shunichi
S
Kato
Eiichi
E
Ishii
D000741:Anemia, Aplastic / Q000097:blood / Q000401:mortality / Q000628:therapy; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000097:blood / Q000401:mortality / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D006086:Graft vs Host Disease / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000401:mortality; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000401:mortality*; D008297:Male; D009369:Neoplasms / Q000097:blood / Q000401:mortality / Q000628:therapy; D011795:Surveys and Questionnaires; D015996:Survival Rate; D016559:Tacrolimus / Q000008:administration & dosage / Q000009:adverse effects; D014184:Transplantation, Homologous; D014280:Triglycerides / Q000097:blood
D007166:Immunosuppressive Agents; D014280:Triglycerides; D001663:Bilirubin; D016559:Tacrolimus