The Enigmatic Langerhan Cell Histiocytosis A Study of Nine Pediatric Cases
Abstract
INTRODUCTION: Langerhan Cell Histiocytosis is a rare disease that is found mostly in children,with an estimated incidence between 0.2 and 2.0 cases per 100,000 children under 15 years of age and a peak incidence between ages of 2-4 years .The disease has preponderance in males ,sometimes as high as 60-70% and is more common in whites of north European descent .The disease encompasses three disorders Eosinophilic Granuloma,Hand Schuler Christian Disease and Letterer Siwe Syndrome according to clinical and pathological features.
AIMS AND OBJECTIVES: Were to establish the clinical spectrum , radiological findings and frequency of bone marrow infiltration in Langerhan Cell Histiocytosis in pediatric population.
PATIENTS AND METHODS: This study included nine children diagnosed with Langerhan Cell Histiocytosis over 15 month period from Feb 2011to Jun 2012 in Children Hospital /ICH Lahore and were referred for bone marrow examination.
RESULTS: All cases presented in the first decade of life with male preponderance(2:1),and mean age being 3.1 years.There were 3 cases of Eosinophilic Granuloma, 2 cases of Hand Schuler Christian Disease and 2 cases of Letterer Siwe disease and 2 cases presented with lymph node involvement only. Anemia was present in all cases , bicytopenia in two and panocytopenia in only one case diagnosed with Letterer-siwe disease. Bone Marrow infiltration was found in only one case diagnosed with Letterer Siwe Disease both on aspirate and trephine biopsy . Radiological examination in 7 out of 9 (77.7%) patients in this study revealed lytic lesions in skull,2 of which had involvement of other bony sites as well.Two cases presented with nodal involvement only.
CONCLUSION: LCH can present in a multitude of ways, The difficulty in diagnosis and the historically complex nomenclature have, as expected made the true prevalence and incidence of these disorders difficult to ascertain therefore it must remain on the differential when a patient presents with osteolytic lesions
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