[Clinical features and diagnosis of langerhans cell hyperplasia]

Abstract

Background & objective: The incidence rate of langerhans cell hyperplasia (LCH) is increasing. Its clinical manifestation is so diversified that it is easy to be misdiagnosed. This study was to analyze its clinical features for the sake of early diagnosis and treatment.

Methods: Clinical data of 44 LCH patients, treated from Jan. 1990 to Dec. 2003 in the First Affiliated Hospital of Sun Yat-sen University, were retrospectively analyzed.

Results: Of the 44 LCH patients, 32 (72.7%) were children, 12 (27.3%) were adults. Of the 32 children patients, 15 (46.9%) were less than 2 years old. The involved organs were bone (20/44, 45.5%), lymph nodes (20/44, 45.5%), skin (16/44, 36.4%), liver and spleen (16/44, 36.4%), lung (14/44, 31.8%), bone marrow (6/44, 13.6%), ear (4/44, 9.1%), eyes (2/44, 4.5%), pituitary gland (2/44, 4.5%), and thymus gland (1/44, 2.3%). Of the 44 patients, 10 had 1 organ involved, 11 had 2 organs involved, 9 had 3 organs involved, 7 had 4 organs involved, 7 had no less than 5 organs involved; 22 children patients and 4 adult patients had multiple organs (liver, lung, bone marrow, and so on) involved; 26 (59.1%) were misdiagnosed as dermatosis (6 cases), hemopathy (7 cases), bone or bone marrow diseases (7 cases), lung tuberculosis (2 cases), diabetes insipidus (2 cases), ear or eye disease (2 cases). Six children patients had bronchopneumonia, and 4 of them had septicemia. Six patients died with 5 cases below the age of 2.

Conclusions: LCH is easy to be misdiagnosed. Children below 2 years old tend to complicate with multiple organ failure (MOF) and bronchopneumonia which have high death rate. Pathology examination is helpful for early diagnosis, treatment, and prevention of severe complications.