Clinical Characteristics and Treatment Outcome of Childhood Langerhans Cell Histiocytosis

Noh, Ha Young; Sohn, Kyoung Ran; Baik, Hee Jo; Kim, Seok Joo; Nam, Ho Song; Kook, Hoon; Hwang, Tai Ju

Abstract

PURPOSE
We analyzed a cohort of patients with Langerhans cell histiocytosis (LCH) to understand the clinical findings, optimal management, and outcome of the disease. METHODS: We performed a retrospective clinical study of LCH from January 1993 to August 2002 at Chonnam National University Hospital. All 39 patients with histologically proven histiocytosis were categorized into Class I (n=22), Class II (n=15) and Class III (n=2) by WHO classification. RESULTS: There were 18 males and 21 females. Mean age at diagnosis was 3.2 years. The common clinical manifestations of Class I were soft tissue swelling, skin rash or nodule, otorrhea; and those of Class II were hepatosplenomegaly, fever, and respiratory symptoms. The most commonly involved organ of Class I was the skeleton; and that of Class II was bone marrow. Abnormal hematologic findings were found in 23 patients, especially in all Class II patients. Infectious etiology was documented in 5 Class II patients (CMV in 3, EBV in 1, mycoplasma in 1). Chemotherapy was given to 19 out of 22 Class I patients. Six of them showed complete remission. Four died during chemotherapy. The overall survival of Class I patients was 78% and that of Class II 63%. Poor prognostic factors of Class I were age < 1 year, over two organ involvement, hemoglobin < 10 g/dL, bilirubin > 1.5 mg/dL. CONCLUSION: The Langerhans cell histiocytosis is a heterogeneous disorder of significant morbidity and mortality. Early recognition and aggressive medical treatment might improve the survival rate.