カンノ ヒトシ
  菅野 仁
   所属   医学部 医学科(東京女子医科大学病院)
   職種   特任教授
論文種別 原著
言語種別 英語
査読の有無 査読あり
表題 Elevated erythrocyte adenosine deaminase activity in a patient with primary acquired sideroblastic anemia.
掲載誌名 正式名:American journal of hematology
略  称:Am J Hematol
ISSNコード:0361-8609(Print)0361-8609(Linking)
巻・号・頁 27(3),pp.216-20
著者・共著者 Kanno H, Fujii H, Tani K, Morisaki T, Takahashi K, Horiuchi N, Kizaki M, Ogawa T, Miwa S
担当区分 筆頭著者
発行年月 1988/03
概要 We report a case of primary acquired sideroblastic anemia (PASA) associated with elevated erythrocyte adenosine deaminase (ADA) activity. The patient was an 85-year-old Japanese male. Analysis of the peripheral blood revealed pancytopenia, and the bone marrow findings showed marked ringed sideroblasts and chromosomal deletion (46XY, 11q-). The erythrocyte ADA activity was 17 times higher than that of normal control, the leukocyte ADA activity was within the normal range, and the plasma ADA activity was 2 times higher than the normal mean. The adenine nucleotides in the patient's erythrocytes were within normal range. According to starch gel electrophoresis, ADA isozyme of the patient was ADA 1. Western blotting showed an increased amount of ADA protein in the patient's erythrocytes. Southern blotting revealed no gene amplification or large structural change. Dot blot analysis of the reticulocyte mRNA showed no increase in the amount of ADA mRNA in the patient's reticulocytes compared with those of reticulocyte-rich controls. We considered that the mechanism of elevated ADA activity in this acquired defect was similar to that found in hereditary hemolytic anemia associated with ADA overproduction.
文献番号 3348207