Clinical Characteristics and Treatment Outcome of Childhood Langerhans Cell Histiocytosis.
- Author:
Ha Young NOH
1
;
Kyoung Ran SOHN
;
Hee Jo BAIK
;
Seok Joo KIM
;
Ho Song NAM
;
Hoon KOOK
;
Tai Ju HWANG
Author Information
1. Department of Pediatrics, Chonnam National University Medical School, Gwangju, Korea. tjhwang@chonnam.ac.kr
- Publication Type:Original Article
- Keywords:
Histiocytosis;
Langerhans cell histiocytosis;
Prognostic factors
- MeSH:
Bilirubin;
Bone Marrow;
Classification;
Cohort Studies;
Diagnosis;
Drug Therapy;
Exanthema;
Female;
Fever;
Herpesvirus 4, Human;
Histiocytosis;
Histiocytosis, Langerhans-Cell*;
Humans;
Jeollanam-do;
Male;
Mortality;
Mycoplasma;
Retrospective Studies;
Skeleton;
Survival Rate;
Treatment Outcome*
- From:Korean Journal of Pediatric Hematology-Oncology
2004;11(1):45-54
- CountryRepublic of Korea
- Language:Korean
-
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.
