Premium
Long‐term complications in uniformly treated paediatric Langerhans histiocytosis patients disclosed by 12 years of follow‐up of the JLSG‐96/02 studies
Author(s) -
Sakamoto Kenichi,
Morimoto Akira,
Shioda Yoko,
Imamura Toshihiko,
Imashuku Shinsaku
Publication year - 2021
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/bjh.17243
Subject(s) - langerhans cell histiocytosis , medicine , histiocytosis , myeloid , mapk/erk pathway , central nervous system , pediatrics , immunology , kinase , biology , disease , microbiology and biotechnology
Summary Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia derived from immature myeloid dendritic cells with the mitogen‐activated protein kinase (MAPK) pathway gene mutation. LCH is rarely fatal, but patients develop various permanent consequences (PCs). We report the frequencies of LCH‐related PCs in paediatric patients ( n = 317) treated by the JLSG‐96/02 AraC‐containing regimens. One‐third of LCH patients had at least one PC at a median follow‐up of 12 years. Central nervous system (CNS)‐related PCs (neurological and endocrinological) accounted for 21·5%, non‐CNS‐related 16·7%. We require novel therapeutic measures to further reduce the frequency of LCH‐related PCs.