SAMUEL I. RAPAPORT, M.D.; EMMETT B. REILLY, M.D.; GURTH CARPENTER, M.D.
Thalassemia 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
RAPAPORT SI, REILLY EB, CARPENTER G. CLINICALLY "INTERMEDIATE" THALASSEMIA DUE TO HYPERSPLENISM COMPLICATING THALASSEMIA MINOR: A CASE REPORT ILLUSTRATING RELIEF OF ANEMIA BY SPLENECTOMY*. Ann Intern Med. 1957;46:1199–1207. doi: https://doi.org/10.7326/0003-4819-46-6-1199
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Published: Ann Intern Med. 1957;46(6):1199-1207.
Hematology/Oncology, Red Cell Disorders.
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