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Published on: May 10, 2017
[Exploring Langerhans cell histiocytosis in childhood: case series]
Irvin Ordoñez-González1, Yail I Chirinos-Chirinos2, Edoardo Figueroa-Pimentel1
1Departamento de Pediatría, Hospital Infantil de México Federico Gómez, Instituto Nacional de Salud, Ciudad de México, México.
Background:
Langerhans cell histiocytosis (LCH) is a rare, multisystem hematologic disease characterized by the clonal proliferation of mononuclear phagocytes in various tissues and organs. In Mexico, its incidence in children is 4.3 cases per million. The disease presents a clinical variety that can mimic other medical conditions, making diagnosis challenging. Pediatricians play a crucial role in the early identification of adenopathy, which can be indicative of LCH or other pathologies.
Clinical Cases:
Case 1. A 6-month-old infant with cervical adenopathy, fever, anemia and cutaneous compromise; biopsy with immunohistochemistry confirmed the disease. Case 2. A 2-year-old boy with retroauricular adenopathy, exophthalmos, and pallor. Lytic bone lesions and lymph node biopsy confirmed the disease, classified as group I. Case 3. A 1-year-old boy with generalized dermatosis, progressive adenopathies, and symptoms of diabetes insipidus; studies confirmed LCH and vasopressin deficiency.
Conclusions:
LCH is currently classified into groups based on the extent of the disease. The presented cases reflect different clinical forms, underscoring the importance of early detection and accurate diagnosis to improve prognosis. Delayed diagnosis can lead to less effective treatments and additional complications. The clinical variability of LCH poses a diagnostic challenge in pediatrics. This analysis emphasizes the importance of early diagnosis and appropriate treatment to improve prognosis. It is essential for pediatricians to consider other causes of adenopathy before considering an oncological diagnosis and to conduct a thorough evaluation for timely referral to subspecialists.

