Multi-system Langerhans cell histiocytosis in a neonate: a case report and literature review

Abstract

Objective To investigate the clinical characteristics of neonatal multi-system Langerhans cell histiocytosis. Methods A female neonate with multi-system Langerhans cell histiocytosis in Guangzhou Women and Children's Medical Center in March, 2013 was reported and related literature was reviewed. The clinical manifestations, and laboratory test, imaging examination and biopsy results were analyzed. Results The neonate had onset of jaundice and rash, then was found to have hepatomegaly, splenomegaly, lymphadenopathy and abnormal blood test. The lymph node biopsy revealed infiltration of Langerhans cells and eosinophils, which were positive for CD1a/S-100 stains. She was diagnosed with multi-system Langerhans cell histiocytosis. Sixty-two cases of neonatal multi-system Langerhans cell histiocytosis were reviewed in the literature, and the most common clinical manifestations included rash, hepatomegaly, respiratory symptoms, imaging changes, abnormal blood test, digestive symptoms and lymphadenopathy. The most frequently involved organs included lung, liver, spleen, bone marrow, bone, gastrointestinal tract, lymph node and mucosa, among which the risky organs (liver, spleen, blood system and lung) accounted for 88.7%(55/62). Conclusions The incidence rate of multi-system Langerhans cell histiocytosis is low in neonates, and it is characterized by multi-system involvement, especially the liver, spleen, blood system, lung and skin. For the neonates diagnosed without intrauterine infections, besides skin biopsy, lymphadenopathy should be dynamically observed and lymph node biopsy should be done earlier so as to establish an early diagnosis. Key words: Histiocytosis, Langerhans-cell; Multiple organ failure; Lymphatic diseases; Infant, newborn