Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sjogren's syndrome (SS) is frequently associated with both reactive and neoplastic lymphoproliferative disease. Over a 12-year period beginning in 1970, 21 of 138 patients with SS followed at two tertiary university medical centers had biopsies taken of enlarged lymph nodes (18) or extranodal lymphoid infiltrates (8). Many had immunologic studies performed on fresh tissue and all had paraffin-embedded tissue available for histochemical and immunoperoxidase studies. Eight of our patients had malignant lymphomas which were chiefly B cell neoplasms including two lymphoplasmacytic lymphomas and two follicular center cell lymphomas. The remaining 13 patients had either reactive adenitis (usually with follicular hyperplasia) or atypical lymphoid hyperplasia which failed to meet both histopathologic and immunopathologic criteria for malignancy. None of the nine patients with reactive hyperplasia has yet progressed to lymphoma, while one of four patients with atypical lymphoid hyperplasia progressed to overt lymphoma. Clinical features such as age, duration of disease, extent of lymphadenopathy, splenomegaly, or parotid swelling failed to identify those subsets of patients with lymphadenopathy at increased risk for lymphoma. Recognition of lymphoma in two patients was greatly facilitated by tissue immunologic studies demonstrating focal areas of monotypic B cell proliferation. In one patient in whom the histopathologic diagnosis was immunoblastic sarcoma of B cells, tumor cells were L26-negative and strongly UCHL1-positive suggesting T cell differentiation. In three patients with relatively homogeneous extranodal lymphoid infiltrates, B cell polyclonality on tissue immunoperoxidase studies, and the absence of cytologic atypia, precluded a diagnosis of malignant lymphoma; none of these three patients has progressed to overt lymphoma. Our results indicate that (1) patients with SS develop a variety of B cell lymphomas and other lymphoproliferative disorders, and (2) the nature of the lymphoproliferative disorder is best determined by multiparameter analysis including immunologic phenotyping.
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PMID:Nodal and extranodal lymphoproliferative disorders in Sjogren's syndrome: a clinical and immunopathologic study. 211 Sep 29

A 39-year-old woman had an adenitis colli followed by anaemia, splenomegaly, atypical lymphoid cells in blood with increased B-lymphocytes, reduced T-suppressor/cytotoxic cells, increased polyclonal IgM, high titres of EB VCA IgG, EB EA IgD&R +/- and EBNA IgG-. The disease progressed slowly for 2 years, splenectomy was followed by clinical improvement; spleen morphology was compatible with a benign disease of viral origin.
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PMID:Chronic infectious mononucleosis; a case report. 225 27

A colony of knockout mice (gene designation Cybb tm1) has been maintained at this institution for 5 years. These mice are lacking the b subunit of NADPH oxidase and are susceptible to experimental infection with Aspergillus fumigatus. The purpose of this study was to document the spontaneous diseases present in these mice which are a murine model of X-linked chronic granulomatous disease and to compare these lesions to those of chronic granulomatous disease in humans. Lesions were documented in 72 mice submitted to the necropsy service. All 72 mice had an acidophilic macrophage pneumonia, and 16 also had lobar suppurative and necrotizing pneumonias caused by Paecilomyces sp. (11 of the 16 mice), A. fumigatus (3 mice), Rhizopus sp. (1 mouse), or Candida guilliermondii (1 mouse). Of the 72 animals, 36 had severe bacterial suppurative and necrotizing to pyogranulomatous pneumonias; lung abscesses yielded cultures of Pseudomonas aeruginosa (n = 3), Enterococcus (n = 6), Staphylococcus aureus (n = 2), S. xylosus (n = 1), coagulase-negative Staphylococcus sp. (n = 4), gram-negative enteric bacilli (n = 6), Klebsiella pneumoniae (n = 1), and Proteus mirabilis (n = 2). Thirteen mice had a necrotizing and suppurative adenitis of the cervical lymph nodes caused by coagulase-negative Staphylococcus sp.; S. aureus, S. xylosus, and S. equorum were recovered from abscesses in the cervical lymph nodes, extremities, and head. Splenomegaly was found in 30 animals and lymphadenopathy in 11 mice. The array of spontaneously occurring infectious diseases and lesions in these mice is similar to that of human patients with chronic granulomatous disease.
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PMID:Pathology of a mouse model of x-linked chronic granulomatous disease. 1221 46