Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighty-nine cases variously diagnosed as histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, or malignant histiocytosis from one institution were reviewed. Follow-up data were obtained for all patients. On the basis of clinicopathologic correlation, the following distinct groups were identified. 1. Disseminated histiocytosis (12 patients) with involvement of more than two organ systems at the time of recognition of the disease, affecting children less than 3 years of age (the patient's general condition is poor and hepatosplenomegaly is common. The patients do poorly, and all the patients in this group of the present study died. 2. Histiocytosis confined to bone, whether monostotic (36 patients) or polyostotic (eight patients). This type is self-healing, does not disseminate, and does not require intensive therapy. Clinically the patients are older and in good general condition during the course of the disease. Histologically histiocytes in these two groups are cytologically benign, and the whole process is inflammatory rather than neoplastic. Presence of giant cells, eosinophils, and necrosis are usually associated with better prognosis. Many neoplastic diseases and infectious processes manifest as histiocytosis. More than a third of the cases of this study diagnosed as histiocytosis proved to be other processes. This confusion contributes to differences of various reports and difficulty of interpretation of findings.
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PMID:Idiopathic differentiated histiocytosis. 30 79

Langerhans cell histiocytosis is a group of idiopathic disorders characterized by the proliferation of cells of the mononuclear phagocyte system and the dendritic cell system. From the clinical point of view there are 3 forms of histiocytosis encountered in child pathology: acute, disseminated called Abt-Letterer-Siwe (it usually affects children under 2 years of age), eosinophilic granuloma and the intermediate clinical form called Hand-Schuller-Christian disease. We present the case of a premature born child that had, even from the day she was born, a rash disseminated on the entire surface of the body associated with enlarged lymph nodes, hepatosplenomegaly and thrombocytopenia. The diagnosis of Langerhans cell Histiocytosis is difficult to establish and this case is an example that confirms it. Therefore, we present the difficulties in diagnosing this case and its particular evolution.
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PMID:[Children's Langerhans cell histiocytosis, diagnostic problems]. 2070 Sep 79