Thalassemia and abnormal hemoglobins in north India (Punjab).
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Biomedical subjects
Publications and source records attributed to S Dash.
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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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Between July 1974 and June 1978 the diagnosis of haemolytic anaemia was made in 267 patients. Thalassaemia major was the leading cause (40-50%) every year except in 1977, when a sharp rise in drug induced haemolysis in G-6-PD deficient patients (61.3%) was encountered. This was possible due to an increase in incidence of vivax malaria and the use of anti-malarial drugs in that year. Abnormal haemoglobins were noted in 9.7% of the total number of patients. Hereditary spherocytosis, autoimmune haemolytic anaemias and paroxysmal nocturnal haemoglobinuria formed a minor cause. Others included copper sulphate poisoning, snake bite, hornet sting and Plasmodium falciparum infection.
Spinal cord compression due to extramedullary haemopoietic tissue in a case of beta thalassaemia and G6 PD deficiency is described. Surgical decompression alone had no beneficial effect. However, the addition of radiotherapy proved very useful.
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An increase in hydrogen ion dissociation was found on sickling due to sickle cell haemoglobin polymerization. Since a decrease in pH favours sickling, this might enhance the sickling process in a vicious cycle.
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