Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neoplasias of the monocyte/macrophage system are rare. They include malignant histiocytosis, sarcomas of dendritic cells, and possibly a malignant form of Langerhans cell histiocytosis. Characterization of all these neoplasms can be easily done along the lines which have been developed for the derivation of the normal cellular counterparts. The possibility of a malignant transformation of the cells of the monocyte/macrophages system is suggested by the fact that all members of this system can undergo mitotic division. The most controversial among the different entities is malignant histiocytosis, which has proved to be anaplastic large cell lymphoma (ALCL) with Ki1 expression in most cases. The few cases of true malignant histiocytosis may or may not express the Ki1 antigen. Sarcomas of dendritic cells have been related mainly to interdigitating or follicular dendritic cells. Prognosis of follicular dendritic cell sarcoma is probably more favorable than that of interdigitating cell sarcoma. Besides these more frequent subtypes, a sarcoma of the sinus lining cells can be separated which represents a low-grade malignant neoplasm. All cases encountered so far were associated with a protracted clinical course, albeit multiple recurrences. A distinctly more aggressive behavior is encountered in those cases which have been identified as a malignant form of Langerhans cell histiocytosis. However, the issue of this particular form of histiocytic disorder is still unsettled.
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PMID:Monocyte/macrophage system and malignancies. 808 13

The T cell immunoglobulin mucin (TIM) proteins are a family of cell surface phosphatidyserine receptors that are important for the recognition and phagocytosis of apoptotic cells. Because TIM-4 is expressed by macrophages and dendritic cells in human tissue, we examined its expression in a range of histiocytic and dendritic cell neoplasms and found moderate to strong immunohistochemical staining in cases of juvenile xanthogranuloma and histiocytic sarcoma, and lower level staining in interdigitating dendritic cell sarcoma, Langerhans cell histiocytosis, acute monocytic leukemia (leukemia cutis), and blastic plasmacytoid dendritic cell neoplasm (hematodermic tumor). TIM-3 was first described on activated T(H)1 cells but was recently shown to also be a phosphatidylserine receptor and mediate phagocytosis. We found TIM-3 was expressed by peritoneal macrophages, monocytes and splenic dendritic cells. We found that it, like TIM-4, is expressed in a range of histiocytic and dendritic cell neoplasms, typically with strong immunohistochemical staining. Cases of diffuse large B cell lymphoma, anaplastic large cell lymphoma, metastatic malignant melanoma, and metastatic poorly differentiated carcinoma generally exhibited negative to minimal heterogenous staining for TIM-4 and TIM-3. We conclude that histiocytic and dendritic cell neoplasms consistently express TIM-3 and TIM-4 and that these molecules are new markers of neoplasms derived from histiocytic and dendritic cells.
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PMID:The phosphatidylserine receptors, T cell immunoglobulin mucin proteins 3 and 4, are markers of histiocytic sarcoma and other histiocytic and dendritic cell neoplasms. 2065 18

Initial extranodal osseous presentation of Classic Hodgkin lymphoma (CHL) is extremely rare. The present study describes a case of CHL in an 11-year-old male presenting with lower back and right hip pain. Radiologic imaging showed a solitary lytic lesion involving the L5 vertebrae and sacrum. Pathologic examination led to a diagnosis of extranodal osseous CHL and further workup proved bone marrow and nodal disease. Diagnosis of extranodal osseous CHL is challenging, especially in this age group and location. When confronted with a solitary lytic bone lesion in a young patient, more common benign and neoplastic entities enter the differential diagnosis and include osteomyelitis, Langerhans cell histiocytosis, epithelioid hemangioma of bone, leukemia, and anaplastic large cell lymphoma (ALCL) among others, with extranodal osseous CHL being one of the less common entities in the differential diagnosis. This case report raises the awareness of extranodal osseous CHL as one of the entities to consider when confronted with a solitary lytic bone lesion in a young patient.
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PMID:Classic Hodgkin lymphoma with initial extranodal osseous presentation: a case report and literature review. 3146 27