Multisystem Langerhans Cell Histiocytosis in Younger Infants First Presenting in Skin: A Case Series

Abstract

Objectives—To investigate the clinical characteristics, managements, outcome, and evaluate the risk factors of Multisystem (MS) Langerhans Cell Histiocytosis (LCH) with diverse skin lesions as the first sign in four young infants. Methods—Their clinical features, disease progression, therapy, and outcomes were reviewed and analyzed retrospectively. Results—The average onset age of skin lesions was about 2 months. Cases 1 and 2 had risk organs involved (RO+) and a lack of bone lesions, and progression could not be reversed by systemic chemotherapy. They both died eventually. Cases 3 and 4 (RO–) had bone involvement and were given systemic chemotherapy for a prolonged duration. Unluckily, Case 3 had a recurrence 2 years later, while Case 4’s recurrence happened nearly one year later, and diabetes insipidus one and a half years later. They both survived and are still in remission. Conclusion—MS-LCH infants with a low age of the first presentation in the skin are prone to dissemination, while RO+ is associated with high mortality. In addition, bone involvement may be a protective factor. Immunohistochemical examination of skin tissue facilitates correct early diagnosis, and adequate follow-up is necessary.

Langerhans Cell Histiocytosis in Children: Literature Review

“…Ранний дебют ГКЛ (в возрасте до 2 лет) ассоциирован с высоким риском развития мультисистемных форм ГКЛ, тогда как моносистемные и монофокальные формы ГКЛ чаще выявляются у детей в возрасте старше 5 лет [4]. Мультисистемная форма заболевания протекает с поражением «органов риска» (печени, селезенки) и недостаточностью гемопоэза и без лечения быстро прогрессирует с развитием полиорганной недостаточности и гибелью ребенка [5]. Популяционная частота ГКЛ, по разным данным, составляет от 2 до 9 случаев на 1 млн детей в год [6,7].…”