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The involvement of the gut by Langerhans cell histiocytosis (LCH) is very rare in adults; however this is usually observed with a disseminated disease in children. We report a 75-year-old male patient who underwent right hemicolectomy for a complicated intestinal diverticular disease. The surgical specimen revealed LCH-like proliferative lesion associated with diverticulitis. The overall morphological and immunohistochemical findings are indistinguishable from LCH. Systemic scans and subsequently performed bone marrow biopsies were free of disease. Although the HUMARA clonality assay cannot be assessed, the lack of evidence of LCH progression or disease elsewhere in the whole body strongly supported the possibility of an atypical reactive phenomenon probably due to the underlying intestinal diverticular disease. Therefore, it is important to avoid diagnosing such a unifocal Langerhans cell proliferation as LCH in patients with underlying pathologies in the absence of systemic involvement. Therefore, without knowledge of clonal status of a unifocal Langerhans cell proliferation, we recommend using the terminology of LCH-like lesion.
Istanbul Faculty of Medicine, Department of Pathology, Istanbul University, Temel Bilimler Binası, Capa, Istanbul, Turkey, firstname.lastname@example.org.
This article was published in the following journal.
Name: Pathology oncology research : POR
Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone.
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General term for the abnormal appearance of histiocytes in the blood. Based on the pathological features of the cells involved rather than on clinical findings, the histiocytic diseases are subdivided into three groups: HISTIOCYTOSIS, LANGERHANS CELL; HISTIOCYTOSIS, NON-LANGERHANS-CELL; and HISTIOCYTIC DISORDERS, MALIGNANT.
A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.
A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder.
Rare malignant neoplasm of dendritic LANGERHANS CELLS exhibiting atypical cytology, frequent mitoses, and aggressive clinical behavior. They can be distinguished from other histiocytic and dendritic proliferations by immunohistochemical and ultrastructure studies. Cytologically benign proliferations of Langerhans cells are called LANGERHANS CELL HISTIOCYTOSIS.
Group of disorders which feature accumulations of active HISTIOCYTES and LYMPHOCYTES, but where the histiocytes are not LANGERHANS CELLS. The group includes HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; SINUS HISTIOCYTOSIS; xanthogranuloma; reticulohistiocytoma; JUVENILE XANTHOGRANULOMA; xanthoma disseminatum; as well as the lipid storage diseases (SEA-BLUE HISTIOCYTE SYNDROME; and NIEMANN-PICK DISEASES).
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...