Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serum beta-2-microglobulin (S-beta 2M) was measured at diagnosis in 44 patients with lymphocytic leukemias and 47 with malignant lymphomas. Among patients with chronic lymphocytic leukemia (CLL) S-beta 2M was raised (greater than 3 mg/l) in 74% and in 23.5% of those with acute lymphoblastic leukemia (ALL). The frequencies for non-Hodgkin's lymphoma (NHL) and Hodgkin's disease (HD) were 59.2% and 40%, respectively. In CLL patients high serum values correlated with large tumor mass, as estimated by Rai's clinical criteria (P less than 0.001), by total peripheral lymphocytes (r = 0.41, P less than 0.05) and by the percentage of bone marrow infiltration of the lymphocytes (P less than 0.01). A significant relation was also found in CLL patients between S-beta 2M level and survival (P less than 0.05). In ALL no association was found between S-beta 2M level with peripheral lymphoblast concentration, French-American-British (FAB) subclassification, splenomegaly, and survival. In NHL patients a significant association was found between S-beta 2M levels and stage of disease (P less than 0.01) and an obscure relation (P less than 0.1) with the presence of lymph nodes greater than 3 cm in diameter, splenomegaly, and hepatomegaly. No significant association was found between S-beta 2M level and histologic subtypes, presence of B symptoms, bone marrow involvement, and survival. In HD patients a significant association was found between the level of S-beta 2M and stage of disease (P less than 0.05) and presence of splenomegaly (P less than 0.05). No association was found between S-beta 2M level and histologic subtypes, lymph nodes greater than 3 cm in diameter, bone marrow involvement, and B symptoms. A significant relation was found between S-beta 2M level and survival in HD patients with widespread disease (P less than .025).
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PMID:Serum beta 2 microglobulin in malignant lymphoproliferative disorders. 388 24

The authors present the characteristics of a group of 23 patients with mantle cell lymphoma. In the group only a slight predominance of men over women was found (1.1:1), the median age was 63 years. Twenty-one (91%) of the patients were diagnosed in stage IV (Ann Arbor). In all these patients the bone marrow was affected. In 19 of them immunoflowcytometric analysis revealed the typical clone of B lymphocytes (CD5 positive)/CD 23 negative). The majority of patients had at the time of diagnosis a large tumourous mass with massive splenomegaly (61%), hepatomegaly (57%) and bulky disease (52%). The node was excised in 17 patients, but in four patients (24%) during the first session the diagnosis was not assessed correctly. In the laboratory findings an inclination to anaemia, thrombocytopenia, lymphocytosis and in particular to high levels of serological indicators of activity of the disease dominated--lactate dehydrogenase, beta-2-microglobulin and serum thymidine kinase. All patients were treated by chemotherapy. Complete remission was achieved by the date of evaluation in one patient (4%), partial remission in seven patients (30%) but 48% patients did not respond to first line treatment. Nine patients of the group died, their median of survival was 14 months (0-24), the median of the follow up of the remaining patients was 133 months (2-31). Two female patients had large-dose treatment with subsequent administration of autologous stem cells. The first one is after 370 days of treatment in complete remission, the second one developed a relapse 100 days after the procedure. From the results and analysis of the literature ensues that mantle cell lymphoma is one of the aggressive malignant B-lymphoproliferations with a very adverse prognosis and it deserves therefore special diagnostic and intense therapeutic attention.
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PMID:[Mantle cell lymphoma as a diagnostic and therapeutic problem]. 1042 28

Hereditary hemochromatosis is an inherited autosomal recessive disease, associated to a mutation in the recently described HFE gene, which is located on the short arm of chromosome 6. The product of this gene combines with the beta-2-microglobulin and the ferritin receptor, and regulates the iron absorption in the small intestine crypt cells. It is possible that the mutation may cause the increased iron uptake by the intestinal cells. The disease is very much common in men after the forties, and its expression is influenced by concomitant alcoholism, iron rich diet, oral and parenteral iron administration, menstrual blood loss or abnormal hemorrhages, blood donations, pregnancy, lactation, and iron malabsorption clinical conditions, like celiac disease. Many patients are asymptomatic, and the diagnosis may be suspected by hepatomegaly of unknown cause, abnormal iron metabolism tests, increased serum aminotransferase levels, diabetes mellitus, and anonymous arthropathy. Less commonly hereditary hemochromatosis presented by symptoms and signs of chronic liver disease, or by the classic triad described by Trousseau skin pigmentation, hepatomegaly and diabetes mellitus. The diagnosis is confirmed by the increased serum ferritin levels and transferrin saturation, and the stainable iron in hepatocytes, measured by scale devised by Scheuer et al, or the measurement of the hepatic iron. The C282Y mutation was found in 64 to 100% of patients; eventually, subjects with hepatic iron overload identical to hereditary hemochromatosis has no mutation, and homozygous for the C282Y mutation do not express iron overload. Iron is best and quickly removed by weekly or twice-weekly phlebotomy of 500 ml, containing approximately 250 mg iron. One to 3 years of weekly phlebotomy may be required to reduce stores to normal. As a guide to long-term maintenance therapy, is recommended phlebotomy every 3 months and the serum ferritin level should be maintained by less than 50 ng/ml.
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PMID:[Hereditary hemochromatosis]. 1217 Feb 86