Premium
Pulmonary Langerhans cell histiocytosis: A variable disease in childhood
Author(s) -
Odame Isaac,
Li Patricia,
Lau Loretta,
Doda Wendy,
Noseworthy Mary,
Babyn Paul,
Weitzman Sheila
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20676
Subject(s) - medicine , langerhans cell histiocytosis , histiocytosis , pediatrics , context (archaeology) , medical record , retrospective cohort study , lung , high resolution computed tomography , radiological weapon , pathological , disease , surgery , biology , paleontology
Background Pulmonary Langerhans cell histiocytosis (PLCH) is rare in childhood but occurs most commonly in children with multisystem (MS) LCH. In adults, by contrast, the lung is the most common and usually the sole organ affected. This retrospective study describes the clinical manifestation, course, and outcome of PLCH in children consecutively diagnosed at two Canadian institutions. Procedure The medical records of children (<18 years of age) consecutively diagnosed with LCH at the two stitutions, were examined to ascertain the demographic details, pathological diagnosis, and organs involved. Further clinical details including, the clinical manifestation, details of therapy, course of lung disease, and clinical outcome were extracted for patients with PLCH. Initial and follow‐up lung radiographs and CT scans were re‐reviewed. Results Of the 178 patients with LCH, 40 (22.5%) presented with MS disease. Thirteen (7.3%) had PLCH, seven at initial diagnosis, and six at the time of disease progression. The median age was 10.1 months and mean was 11.9 months at diagnosis of PLCH. Lung involvement was always in the context of MS LCH, and half of the patients had no respiratory symptoms. Disease‐free survival was around 70%, with a mean follow‐up duration of 7 years. Of the four patients who died, three had other risk‐organ involvement. Five of the nine surviving patients have had complete radiological resolution of PLCH. Conclusion PLCH is seen in less than 10% of childhood LCH, but more than 30% of MS LCH. About half of children with PLCH may be asymptomatic, and the prognosis appears to depend on the presence or absence of other risk‐organ involvement. The MS PLCH found in children appears to be a different disease from the single system (SS) PLCH seen in adults. © 2005 Wiley‐Liss, Inc.