Clinically intermediate thalassemia due to hypersplenism complicating thalassemia minor; a case report illustrating relief of anemia by splenectomy.

Abstract

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. SturgeonItanoValentine PHAWN: Chronic hemolytic anemia associated with thalassemia and sickling trait, Blood 7: 350-357, 1952. CrossrefMedlineGoogle Scholar6. SilvestroniBianco EI: Genetic aspects of sickle cell anemia and microdrepanocytic disease, Blood 7: 429-435, 1952. CrossrefMedlineGoogle Scholar7. SingerSingerGoldberg KLSR: Studies of abnormal hemoglobins. XI. Sickle cell-thalassemia disease in the Negro. The significance of the S + A + F and S + A patterns obtained by hemoglobin analysis, Blood 10: 405-415, 1955. CrossrefMedlineGoogle Scholar8. SingerKrausSingerRubinsteinGoldberg KAPLHMSR: Studies on abnormal hemoglobins. X. A new syndrome: hemoglobin C-thalassemia disease, Blood 9: 1032-1046, 1954. CrossrefMedlineGoogle Scholar9. ZuelzerKaplan WWE: Thalassemia-hemoglobin C disease. A new syndrome presumably due to the combination of the genes for thalassemia and hemoglobin C, Blood 9: 1047-1054, 1954. CrossrefMedlineGoogle Scholar10. ChernoffMinnichChongchareonsuk AIVS: Hemoglobin E, a hereditary abnormality of human hemoglobin, Science 120: 605-606, 1954. CrossrefMedlineGoogle Scholar11. SturgeonItanoBergren PHAWR: Clinical manifestations of inherited abnormal hemoglobins. I. The interaction of hemoglobin-S with hemoglobin-D. II. Interaction of hemoglobin-E and thalassemia trait, Blood 10: 389-404, 1955. CrossrefMedlineGoogle Scholar12. Smith CA: Detection of mild types of Mediterranean (Cooley's) anemia, Am. J. Dis. Child. 75: 505-527, 1948. MedlineGoogle Scholar13. MinnichNakornChongchareonsukKochaseni VSSS: Mediterranean anemia. A study of thirty-two cases in Thailand, Blood 9: 1-23, 1954. CrossrefMedlineGoogle Scholar14. LichtmanWatsonFeldmanGinsbergRobinson HCRJFVJ: Studies on thalassemia. Part I. An extracorpuscular defect in thalassemia major. Part II. The effects of splenectomy in thalassemia major with an associated acquired hemolytic anemia, J. Clin. Investigation 32: 1229-1235, 1953. CrossrefMedlineGoogle Scholar15. SmithSchulmanAndoStern CHIREG: Studies in Mediterranean (Cooley's) anemia. I. Clinical and hematologic aspects of splenectomy, with special reference to fetal hemoglobin synthesis, Blood 10: 582-599, 1955. CrossrefMedlineGoogle Scholar16. WolmanDickstein IJB: Changing concepts in Mediterranean (Cooley's) anemia, Am. J. M. Sc. 212: 723-737, 1946. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Received for publication February 7, 1956.From the Medical and Pathology Services, Veterans Administration Hospital, Long Beach, California.Requests for reprints should be addressed to Samuel I. Rapaport, M.D., Dept. of Medicine, University of California Medical Center, Los Angeles 24, Calif. 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 ...