Analysis of clinical manifestations and treatment of pulmonary Langerhans cell histiocytosis:A 17 cases report
LU Shifeng
FANG Yongjun
RUI Yaoyao
HE Lulu
ZHOU Li
HUANG Jie
WU Peng
LIN Rufeng
WANG Yongren
LU Qin
Abstract：Objective To study the characteristic clinical manifestations, CT manifestations, diagnosis and treatment charac?teristics, and analyze its relationships with prognosis of the children with pulmonary Langerhans cell histiocytosis( PLCH) . Methods In a retrospective study, the clinical features and imaging data of 17 children with PLCH were analyzed as well as the efficacy and prog?nosis after typeⅢchemotherapy of 6?week induction phase and 52?week maintenance phase. Results All children suffered onset pul?monary diffuse change with multiple organ damage and in the process of disease development;most of the chest computed tomography ( CT) showed characteristic changes. All the 17 children were received typeⅢchemotherapy, and 8 cases got complete remission with good prognosis, 4 cases relapsed after chemotherapy including 1 case with good prognosis and 3 cases of death, 3 cases abandoned treatment due to progression during chemotherapy. In addition, 1 case with progression received re?induction chemotherapy and survived with the present continuous chemotherapy. One case of maintenance chemotherapy treatment was followed?up. Conclusion Children with PLCH were infants and young children with multiple organ damage. Early intenstive chemotherapy can improve the prognosis, but some cases of lung progress are apparent.
Keywords：ChildrenPulmonary Langerhans cell histiocytosisClinical analysis
Publication Date：2014-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：3( 1135-1137 )