Clinical Analysis of 25 Cases of Langerhans Cell Histiocytosis / 实用儿科临床杂志

ABSTRACT

Objective To explore the clinical and laboratory characteristics of langerhans cell histiocytosis (LCH) in children, so as to improve diagnosis level and decrease misdiagnosis rate.Methods Twenty-five cases of LCH from Jan.1996 to Feb.2006 were analyzed by retrospective study. The clinical data were collected and abstracted for information regarding clinical symptom, physical sign, laboratory examination, imaging,pathology,diagnosis and treatment.Results Some laboratory findings in hemogram, bone marrow examination and chest X-ray were non-specific.The X-ray characteristic of skeleton was osteolysis. Computerized tomography and magnetic resonance imaging were important in defining the extent of lesion in fundus cranii and sella.Seven cases were examined for anti-Epstein-Barr virus(EBV) IgM, 3 cases were positive;5 and 3 cases out of 10 cases showed humoral and cellular immunity abnormality,respectively. The misdiagnosis rate was 52%,1 case had been misdiagnosed for 7 years. Chemotherapy was effective in the future.Conclusions The clinical manifestations of LCH varies widely, leading to high rate of misdiagnosis.The etiology of LCH is unclear,and some of our patients show the evidence of EBV infection or immunity abnormality. Definitive diagnosis of LCH is based on pathology. Ultrastructure and immunophenotype should be done to improve diagnosis level.