Langerhans Cell Histiocytosis Diagnosed at the Neonatal Period / 임상소아혈액종양

ABSTRACT

BACKGROUND: We retrospectively analyzed the clinical data to describe the characteristics of lesions, extent of disease, and outcomes in patients with neonatal presentation of Langerhans cell histiocytosis (LCH). METHODS: Clinical data obtained from neonate diagnosed with LCH at Asan Medical Center between March 1998 and May 2010 were studied. A review of the medical records, analyzing the clinical parameters such as age when signs and symptoms initially manifested, morphologic characteristics of lesions, age at diagnosis, additional organ involvement, therapy administered, course, and outcome, including delayed sequelae was done. RESULTS: Among seventy nine (36 female, 43 males) patients, 6 patients (3 female, 3 male) were presented symptoms within 4 weeks after birth and diagnosed with neonatal LCH (7.6%). Median age of the 6 patients was 11.5 (5-42) days and LCH was diagnosed based on the CD1a positivity. At diagnosis, 4 patients showed single system (SS) LCH (66.7%) whereas 2 patients had multisystem (MS) LCH (33.3%) and skin lesions were the most common initial manifestation (83.3%). The probability of event free survival at 12 years was 83.3%. CONCLUSION: Skin lesion was the most prominent manifestation of the neonatal LCH with complete spontaneous involution. A multi-organ evaluation at the time of diagnosis is important to ascertain the possibility of multisystem involvement with LCH.