Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare instance of IgG-kappa monoclonal gammopathy in a patient with beta-thalassaemia trait is reported. The patient had a smoldering multiple myeloma with no apparent clinical symptoms pertaining to the disease and was a non-responder to conventional Melphalan and prednisolone therapy. Beta-thalassaemia trait was detected while investigating him for anaemia. A state of altered immunological reactivity, chronic infections and associated biliary tract disease are some of the pathogenetic mechanisms suggested. However in this patient none of these were operative.
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PMID:Monoclonal gammopathy in beta thalassaemia. 308 4

We have studied both the humoral and cell mediated immune systems of 23 children with beta-thalassaemia major. In children who had not been splenectomized, a 3-fold expansion in the number of circulating B cells and a modest polyclonal gammopathy was present. Of these patients 70% had decreased numbers of circulating T4 cells; 83% were unresponsive to skin testing with Candida albicans, and the majority had decreased lymphocyte proliferative responses in vitro. In children who had been splenectomized, there was a 10-fold increase in the number of circulating B lymphocytes and a 2-fold increase in the number of T4 and T8 cells present in peripheral blood. Additionally, these patients as a group were more responsive to both skin testing and lymphocyte stimulation in vitro with Candida albicans. Seven patients had an inverted T4/T8 ratio. One child has positive serology to HIV by ELISA and Western Blot techniques with a normal T4/T8 ratio. Thus, while children with thalassaemia are at risk for exposure to HIV, the immunological abnormalities associated with the disease and/or its treatment necessitates cautious interpretation of any AIDS-related immunological changes.
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PMID:Abnormalities in the immune system of children with beta-thalassaemia major. 349 80