CLINICALLY "INTERMEDIATE" THALASSEMIA DUE TO HYPERSPLENISM COMPLICATING THALASSEMIA MINOR: A CASE REPORT ILLUSTRATING RELIEF OF ANEMIA BY SPLENECTOMY
Authors: S I RAPAPORT, E B REILLY, G CARPENTER
Journal: Annals of Internal Medicine (1957)

Case Reports1 June 1957CLINICALLY "INTERMEDIATE" THALASSEMIA DUE TO HYPERSPLENISM COMPLICATING THALASSEMIA MINOR: A CASE REPORT ILLUSTRATING RELIEF OF ANEMIA BY SPLENECTOMYSAMUEL I. RAPAPORT, M.D., EMMETT B. REILLY, M.D., GURTH CARPENTER, M.D.SAMUEL I. RAPAPORT, M.D.Search for more papers by this author, EMMETT B. REILLY, M.D.Search for more papers by this author, GURTH CARPENTER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-46-6-1199 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptThalassemia major and thalassemia minor are well recognized hematologic syndromes due to different degrees of an inherited defect in hemoglobin synthesis.Thalassemia major is a severe childhood anemia (Cooley's anemia, Mediterranean anemia) with massive hepatosplenomegaly, jaundice from accelerated hemolysis of defective red cells, and bony changes secondary to an expanded, hyperplastic marrow. The red cells are hypochromic, microcytic and abnormally resistant to hypotonic saline. Target, oval and stippled red cells and normoblasts characterize the peripheral blood smear. The hemoglobin migrates closely with normal adult hemoglobin (hemoglobin A) on filter paper electrophoresis, but alkali denaturation reveals large amounts of slowly denaturing...Bibliography1. SingerChernoffSinger KAIL: Studies on abnormal hemoglobins. 1. Their demonstration in sickle cell anemia and other hematologic disorders by means of alkali denaturation, Blood 6: 413-428, 1951. CrossrefMedlineGoogle Scholar2. ValentineNeel WNJV: Hematologic and genetic study of the transmission of thalassemia (Cooley's anemia, Mediterranean anemia), Arch. Int. Med. 74: 185-196, 1944. CrossrefGoogle Scholar3. MarchSchlyenSchwartz HWSMSE: Mediterranean hemopathic syndromes (Cooley's anemia) in adults. Study of a family with unusual complications, Am. J. Med. 13: 46-57, 1952. CrossrefMedlineGoogle Scholar4. PowellRodarteNeel WNJGJV: The occurrence in a family of Sicilian ancestry of the traits for both sickling and thalassemia, Blood 5: 887-897, 1950. CrossrefMedlineGoogle Scholar5. 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PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThalassemia-hemoglobin E disease in turkey, with hypersplenism in one case 1 June 1957Volume 46, Issue 6Page: 1199-1207KeywordsAnemiaAttentionBeta-thalassemiaCellsHemoglobinHepatosplenomegalyPaper electrophoresisResearch laboratoriesSplenectomyThalassemia ePublished: 1 December 2008 Issue Published: 1 June 1957 PDF downloadLoading ...