Unstable Hemoglobinemia, Hb Buenos Aires, Bryn Mawr, Followed Up for Eighteen Years

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  • 不安定ヘモグロビン症Hb Buenos Aires,Bryn Mawr : 18年の経過観察
  • Case Report: Unstable Hemoglobinemia, Hb Buenos Aires, Bryn Mawr, Followed Up for Eighteen Years

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A 20-year-old man has been under observation for 18 years because of unstable hemoglobinemia, Hb Buenos Aires, Bryn Mawr (fl-globin, Phe85Ser). At the age of 19 years, he was hospitalized because of fever and hemolytic crisis, and the symptoms resolved after infusion of antibiotics. Nucleotide sequencing of the fl-globin gene confirmed that the patient was heterozygous for the mutation. The patient's erythrocytes showed an increased affinity for oxygen and a prolonged glycerol lysis time. We review a previously reported single family and 5 other cases, and discuss the clinical significance of splenectomy and plasma-derived haptoglobin.

Journal

  • 福岡醫學雜誌

    福岡醫學雜誌 95 (11), 297-304, 2004-11-25

    Fukuoka Medical Association

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