Langerhans cell histiocytosis; an institutional study

Abstract

Background: Langerhans cell histiocytosis is a complex rare disease of the dendritic cell system.The disease can occur at any age, with a peak at 5-10 years. Here, we present the spectrum of this uncommon disease with different sites of involvement and its histopathological differential diagnosis. Material and Methods: Study included 24 cases of Langerhans cell histiocytosis out of approximately 50,000 biopsies received in the department.Results: The male to female ratio was 1.6:1 with mean age of 12 years. Commonest site of involvement was skin 37.5%, followed by bone 33.4%. Lymph nodes 20.9%, bone marrow 4.1% and thyroid 4.1% involvement was seen. Among the 9 cases of bone involvement, commonest site was humerus (3 cases), sternum (2 cases) and one case each in frontal bone, fibula, temporal bone and rib.Conclusion: Langerhans cell histiocytosis should be considered in the differential diagnosis of histiocytic lesions involving various organs, especially in childhood.

Clinical Features and Outcome in Children With Langerhans Cell Histiocytosis. A Single Institution Experience From Pakistan

“…3 In our study there were only 29 patients diagnosed and treated with LCH during 10 years and most of our patients were within the ages of 2-10 years (58%). Similar results have been reported by Shovana et al 10 , in which the male to female ratio was 2.6:1 with male predominance similar to literature. 10,3 LCH is a non-hereditary disorder and usually sporadic, but also has some genetic predisposition which may play a role in its development as about 1% of patients have relatives with LCH, monozygotic twin pairs are concordant for LCH.…”

“…10,3 LCH is a non-hereditary disorder and usually sporadic, but also has some genetic predisposition which may play a role in its development as about 1% of patients have relatives with LCH, monozygotic twin pairs are concordant for LCH. 10,9 In the present study there was one monozygotic twin pair who presented with MS-LCH with risk organ involvement.…”