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)

We report an autopsy case of true malignant histiocytosis that developed during chemotherapy for mediastinal immature teratoma. The patient was a 14-year-old boy who exhibited hepatosplenomegaly while receiving chemotherapy for a mediastinal immature teratoma that had been resected 11 months before. The spleen and liver of the excisional biopsy displayed infiltration of multinucleated giant atypical cells with prominent erythrophagia in massive aggregations. These atypical cells expressed CD68, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, and vimentin, suggesting that the tumor cells may have been derived from macrophages. Immunocytochemistry showed p53 expression in the tumor cells of the malignant histiocytosis, as well as in the elements of the immature teratoma. Direct sequence analysis showed the p53 mutation in the tumor cells of the immature teratoma to be a mutation at codon 175 (exon 5), whereas the mutation in the malignant histiocytosis occurred at codon 285 (exon 8), ie, polyclonality was exhibited and these features suggested that the malignant histiocytosis arose independently from the immature teratoma during the chemotherapy.
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PMID:True malignant histiocytosis developed during chemotherapy for mediastinal immature teratoma. 889 99

We report here an autopsy case of true malignant histiocytosis. The patient was a 67-year-old woman who exhibited fever, wasting, hepatosplenomegaly, and progressive pancytopenia. The bone marrow aspiration disclosed hemophagocytosing cells, which resembled histiocytes. The molecular analysis did not show the clonal gene rearrangement of T-cell receptor or immunoglobulin heavy chain. Although the patient had been started on methylprednisolone pulse therapy and chemotherapy with etoposide, she died from cerebral hemorrhage. The autopsy specimens of spleen and liver showed extensive infiltration of atypical cells, for which histiocytic origin was identified with an immunohistochemical method using monoclonal antibodies against CD11c, CD68, macrophage colony-stimulating factor (M-CSF), M-CSF receptor, lysozyme, antitrypsin and alpha1-antichymotrypsin. Recent investigations have disclosed that in most cases diagnosed as malignant histiocytosis, hemophagocytosis was reactive and not evoked by histiocytic malignancy. True malignant histiocytosis, for which histiocytic origin is confirmed, is extremely rare.
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PMID:A case of true malignant histiocytosis: identification of histiocytic origin with use of immunohistochemical and immunocytogenetic methods. 1202 39