Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purine metabolic enzymes adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), and 5'nucleotidase (5NT) play an important role in normal lymphocyte differentiation. Abnormal levels of one or all of these enzymes have been associated with immunodeficiency diseases and lymphoproliferative disorders. ADA, PNP, and 5NT activity was measured in peripheral blood T cells from 24 patients with Hodgkin disease (HD) (12 in complete remission and 12 with active disease) to determine whether an association existed between enzyme abnormalities and the decreased cellular immune function previously described in this disorder. HD patients had a significantly decreased absolute lymphocyte count (1,618 +/- 1107/mm3; mean +/- SD) compared to controls (2,320 +/- 980; p less than .001). ADA, PNP, and NT activity was assessed in lymphocyte extracts by measuring the conversion of radiolabeled substrates to products over time. ADA activity expressed as mean +/- SEM nanomoles/10(6) lymphocytes/hr was significantly decreased in T cells from HD patients (84.6 +/- 7.5) compared to controls (128 +/- 12.3; p less than 0.025). Likewise, 5NT was significantly decreased in HD patients (12.7 +/- 1.3) compared to controls (24.0 +/- 3.6; p less than .005). There was not a significant difference in PNP activity between both groups. Low 5NT activity was present irrespective of whether patients had active disease (12.1 +/- 1.5) or were in unmaintained complete remission (14.5 +/- 2.4). These findings suggest that biochemical abnormalities may be responsible for or related to the persistent abnormalities in T-cell function noted throughout the clinical course of HD.
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PMID:Decreased adenosine deaminase (ADA) and 5'nucleotidase (5NT) activity in peripheral blood T cells in Hodgkin disease. 301 Jul 5

Large-cell non-Hodgkin's lymphomas (T- and B-immunoblastic, centroblastic and true histiocytic lymphomas) have a heterogeneous clinical course. In the present study the clinical and morphological data of 20 cases of histiocytic sarcoma (true histiocytic lymphoma) are presented. Diagnosis was supported by immunohistochemistry, cytochemistry, rosette assays and/or electron microscopy. Although the follow-up was relatively short (up to 144 months, mean 26 months), the clinical data differed clearly from the series of large-cell non-Hodgkin lymphomas, recorded in the literature. Differences were found in age distribution with a peak in the third decade, in organ involvement showing a preference for skin, gastrointestinal tract and bone, and in response to therapy. In general, histiocytic sarcoma appears to have a more favourable response to therapy and clinical course than the other large-cell lymphomas (T- and B-immunoblastic and centroblastic lymphomas). Moreover, preliminary observations in the group of histiocytic sarcomas suggested that the presence of lysozyme and/or 5-nucleotidase and the absence of alpha 1-antitrypsin in the cytoplasm is associated with a better response to therapy and favourable clinical course.
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PMID:Histiocytic sarcoma (true histiocytic lymphoma): a clinicopathological study of 20 cases. 632 97