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- W2912496569 abstract "Langerhans Cell Histiocytosis (LCH) is a rare disorder characterized by proliferation and infiltration of abnormal dendritic cells and/or macrophages into various organs and tissues. Gastrointestinal tract involvement is extremely rare. We present a very unusual case of isolated colonic LCH incidentally found during routine screening colonoscopy. A 60-year-old male presented for routine screening colonoscopy. He denied gastrointestinal complaints including abdominal pain, nausea, vomiting, diarrhea, weight loss, blood in the stool or change in bowel habits. His medical history was only significant for heavy smoking leading to the development of the chronic obstructive pulmonary disease. Review of systems was only significant for arthralgias in knees and bilateral cervical lymphadenopathy. His vital signs were normal. Physical exam was essentially unremarkable. Basic laboratory data including complete blood count, the metabolic panel, and liver profile were within normal limits. Colonoscopy revealed innumerable prominent 3-5 mm nodular lesions throughout the colon, more prominent to the transverse and descending colon. Pathology showed diffuse aggregates of histiocytes in the lamina propria of the mucosa. Immunostains for CD1a were strongly positive within the histiocytes confirming the diagnosis of LCH. LCH is usually seen in young children and it is rare in adults. Clinical presentation depends upon the sites of Langerhans cells infiltration. Most common sites involved are skin, bones, lungs, lymph nodes, bone marrow, spleen, and liver. Pathogenesis of this condition is not well understood and there are debates regarding whether it is a reactive or neoplastic process. Ten cases of isolated gastrointestinal LCH have been reported in the literature to our knowledge. Histiocytic disorders are classified into 3 groups: (1) Dendritic cell histiocytosis (2) macrophage-related disorders and (3) malignant histiocytosis. LCH belongs to the group (1). More commonly children present with lytic bone lesions and skin lesions. About 2% of patients with gastrointestinal tract involvement present with diarrhea or malabsorption syndrome. Diagnosis of LCH is challenging and requires identification of Langerhans cells along with immunohistochemical confirmation with CD1a, S100, and CD 207 staining. It must be distinguished from other hematopoietic disorders as prognosis and management vary. LCH is treated with systemic steroids and chemotherapy with variable responses.Figure: Nodular lesions of the colonic mucosa.Figure: Nodular lesions of the colonic mucosa (NBI view).Figure: Diffuse aggregates of histiocytes in the lamina propria." @default.
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- W2912496569 date "2017-10-01" @default.
- W2912496569 modified "2023-09-25" @default.
- W2912496569 title "Langerhans Cell Histiocytosis of the Colon: A Rare Entity" @default.
- W2912496569 doi "https://doi.org/10.14309/00000434-201710001-01486" @default.
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