Retrospective analysis of non-anaplastic peripheral T-cell lymphoma in pediatric patients in Japan.
BACKGROUND
Reports of non-anaplastic peripheral T-cell lymphoma (PTCL) in pediatric patients are relatively rare.


METHODS
We performed a retrospective analysis in patients with PTCL over an 18-year period (1991-2008).


RESULTS
We could analyze clinical data in 21 patients with non-anaplastic PTCL; 10 were female and 10 male. Median age of onset was 11 years (range: 1-21 years). There were nine patients with PTCL, not otherwise specified (PTCL-NOS); ten with extranodal NK/T-cell lymphoma, nasal type; one with angioimmunoblastic T-cell lymphoma; and one with subcutaneous panniculitis-like T-cell lymphoma. Initial lesions involved cervical lymph nodes in five patients, and the skin in five patients. In five patients, hemophagocytic syndrome (HPS) was the initial clinical feature. There were 12 patients with advanced stage disease (stages III and IV). Chemotherapy and radiation was administered in 18 and 2 patients, respectively. Among the two patients who did not receive chemotherapy and radiation, one patient died while being treated for HPS but another improved spontaneously. Although 5 patients relapsed, 18 of 21 patients remained alive without disease at last follow-up. Five-year overall survival rate was 85.2%.


CONCLUSIONS
Generally, the outcome results of conventional chemotherapy for high-risk PTCL are poor in adult patients. However, the excellent results in our study suggest that PTCL of childhood is quite different from that of adulthood. Although this study is first report about PTCL of Asian children, the number of patients was small in this study. Larger studies are needed to confirm these findings.
Kobayashi
Ryoji
R
Department of Pediatrics, Sapporo Hokuyu Hospital, Shiroishiku, Sapporo, Japan. r-koba@jacls.jp
Yamato
Kazumi
K
Tanaka
Fumiko
F
Takashima
Yoshifumi
Y
Inada
Hiroko
H
Kikuchi
Akira
A
Kumagai
Masa-Aki
MA
Sunami
Shosuke
S
Nakagawa
Atsuko
A
Fukano
Reiji
R
Fujita
Naoto
N
Mitsui
Tetsuo
T
Tsurusawa
Masahito
M
Mori
Tetsuya
T
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D016411:Lymphoma, T-Cell, Peripheral / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D012189:Retrospective Studies; D033581:Stem Cell Transplantation; D015996:Survival Rate; D055815:Young Adult