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01.Sickle Cell Hemoglobinopathy And Sudden Death (pp. 125-130)
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Multiple Myeloma In Sickle Cell Syndromes (pp. 113-124) $25.00
Authors:  Andreas, Kaloterakis and Anna, Filiotou (University of Athens School of Medicine, Second Department of Medicine at Hippocration Hospital, Athens, Greece)
Abstract:
Multiple myeloma (MM) has rarely been reported in patients with sickle cell syndromes (SCS). We describe seven Greek sickle cell patients ranging in age from 42 to 65-years, five sickle cell-thalassaemia double heterozygotes, one sickle cell disease homozygote (HbSS), and one sickle cell heterozygote (SA), who developed MM (three M-IgG., one M-IgG., one M-IgA., one M-IgG..IgA. (biclonal) and one Bence Jones-. (BJ-.). Two of these seven patients also developed amyloidosis (AL). Six of the seven cases represent the first documented association of MM with HbS▀+ thalassaemia or HbSA. Generalized bleeding diathesis, stroke, grand mal seizures, bone marrow necrosis and other complications of whole blood hyperviscosity syndrome due to serum hyperviscosity aggravated by sickle cell vaso-occlusion, were treated by plasmaphereses, exchange transfusions and/or blood transfusions. Due to the current availability of medical facilities, increased survival in patients with SCS may have an impact on the incidence of MM among such patients, if a link does exist between the two diseases. Six of our patients had chronic hemolysis, cholelithiasis - chronic billiary tract infection which may predispose one to MM; two of these patients progressed from monoclonal gammopathy of undetermined significance (MGUS) to MM. We believe that further confirmatory studies are required in order to define the relationship, if any, between SCS and MM. 


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Multiple Myeloma In Sickle Cell Syndromes (pp. 113-124)