Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 20-year-old black man with known sickle cell anemia was admitted to the hospital with the signs and symptoms of right middle lobe pneumonia. Because of a hemoglobin value of 3.9 g/dL, he received a transfusion of 3 units of packed erythrocytes. Seven days later, hypertension, headache, and one grand mal seizure occurred. Evaluation of his condition gave normal findings. Similar events, some terminating in cerebral hemorrhage and death, have been reported in patients with hemolytic anemia after they received multiple transfusions. To our knowledge, this patient represents the first adult with sickle cell anemia who had this syndrome. Prompt antihypertensive therapy may have been of benefit.
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PMID:Hypertension and a seizure following transfusion in an adult with sickle cell anemia. 670 31

Multiple myeloma (MM) is rare among patients with sickle cell syndromes (SCS). We describe six Greek sickle cell patients aged 56 to 65 years: five haemoglobin Sbeta+thalassaemia (HbSbeta+ (thal), one sickle cell anaemia (HbSS), who developed MM (three IgGkappa, one IgGlambda, one IgAkappa, and one IgGK-IgAK (biclonal). Our HbSbeta+thal cases, represent the first reported association of this entity with MM. Generalized bleeding diathesis, stroke, grand mal seizures, bone marrow necrosis and other clinical manifestations due to hyperviscosity aggravated by sickle cell vasoocclusion were treated by plasmaphereses and exchange blood transfusions. The increase of mean survival in SCS patients due to the current medical facilities may have an impact on the incidence of MM among them, if a pathogenetic link between the two conditions exists. All our patients carried a diagnosis of cholelithiasis which may predispose to MM; two of them progressed from a monoclonal gammopathy of undetermined significance (MGUS) to MM. Further studies are needed in order to understand the relationship between SCS and MM.
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PMID:Multiple myeloma in sickle cell syndromes. 1158 27