American journal of hematology
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The Indian rubber estate workers in Negri Sembilan, Malaysia, who originated from Orissa in India were found to have a high frequency of Hb S (Joishy SK, Hassan K: Clin Res 28:280, 1980). Unlike the usually severe clinical picture of sickle cell anemia seen in African and American blacks, the clinical picture of the disease in this population was mild and many have reached old age. We studied the leukocyte DNA of 12 patients with sickle cell anemia, ranging in age from 4 to 61 years and 30 sickle cell trait carriers, ranging in age from 7 to 63 years, for the presence of alpha-globin gene deletions by gene mapping according to Southern (Southern EM: J Mol Biol 98:503, 1975), using alpha- and zeta-globin gene probes obtained by nick translation of the alpha- and zeta-globin genes cloned into plasmid. ⋯ None of the sickle cell anemia patients and Hb S trait carriers had deletion type alpha-thal1. The sickle cell anemia patients had very high levels of Hb F and low levels of Hb A2. The Hb S trait carriers with alpha-thal2 had relatively low levels of Hb S.
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A 69-year-old man with immunoblastic lymphadenopathy and autoimmune hemolytic anemia who had no previous exposure to blood products developed a severe febrile nonhemolytic transfusion reaction following the initial infusion of packed red blood cells. The reaction recurred with transfusion of packed red blood cells, but not when freeze-thawed red blood cells were used. ⋯ The granulocyte antibodies fluctuated in titers with disease activities, and could be completely removed from the serum by autologous granulocyte absorption. We conclude that our patient had granulocyte autoantibodies which probably produced febrile transfusion reactions.
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Four patients with nonhematologic malignancies had the simultaneous finding of hemolytic anemia due to high-titer cold agglutinins. In each patient, the cold agglutinin had "anti-I" specificity and was of the IgM kappa immunoglobulin class. Although patients with hematologic malignancies not uncommonly have cold agglutinins, the association between these antibodies and nonhematologic malignancies is unusual.
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In summary, LAP is an intriguing enzyme and its control is related to pituitary-adrenal function. A review of the changes in LAP activity which occur in some physiological conditions and in disease states has been presented. The function of LAP, however, is unknown. ⋯ Of prime importance is the differentiation of CML from a leukemoid reaction or agnogenic myeloid metaplasia with a leukocytosis. However, in no instance is the LAP value alone diagnostic of any disease. It remains a laboratory test to be utilized in conjunction with all other available clinical data.
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Whether the trimodality in the relative concentration of the hemoglobin variant Hb Leslie in heterozygotes (Huisman, Hemoglobin 1:349-382, 1977) is due to a polymorphism of the alpha-chain structural genes was investigated by conventional incubation of reticulocytes with 14C-leucine. In addition, an aliquot from each of the incubations was incubated under the same conditions but without isotope. ⋯ A concomitant alpha-chain deficiency results in a reduced incorporation of betaLeslie chains into the tetrameric Hb Leslie molecules, while the quantity of Hb Leslie produced correlates with the degree of alpha-chain deficiency. Excess of betaLeslie chains is preferentially degraded.