Pediatric pulmonary multisystem langerhans cell histiocytosis: does lung lesion severity affect the outcome?

ABSTRACT:

Background

The pediatric pulmonary multisystem Langerhans cell histiocytosis (PPM LCH) is associated with either low risk or high risk organ(s). The nodulo-cystic lung lesions although pathognomonic, yet are very variable in severity and remain a source of controversy in certifying pulmonary LCH diagnosis. The study aimed to examine the prognostic value of clinical respiratory manifestations and radiological lung lesions severity. This is through associating a CT chest triad of bilateral, extensive and diffuse lesions. It is a retrospective study of 350 LCH patients who received systemic treatment at Children's Cancer Hospital Egypt during the period from 2007 to 2020.

Results

Sixty-seven patients (67/350-19.1%) had PPM LCH at presentation. Severe lung lesions were present i

SUBMITTER: Sedky M 

PROVIDER: S-EPMC10655418 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature