Elevated erythrocyte adenosine deaminase activity in a patient with primary acquired sideroblastic anemia

✍ Scribed by Hitoshi Kanno; Hisaichi Fujii; Kenzaburo Tani; Takayuki Morisaki; Keisuke Takahashi; Naomi Horiuchi; Masahiro Kizaki; Tetsuhei Ogawa; Shiro Miwa

• Publisher: John Wiley and Sons
• Year: 1988
• Tongue: English
• Weight: 416 KB
• Volume: 27
• Category: Article
• ISSN: 0361-8609
• DOI: 10.1002/ajh.2830270313

✦ Synopsis

We report a case of primary acquired slderoblastic anemia (PASA) associated with elevated erythrocyte adenosine deaminase (ADA) activity. The patient was an 85-yearold Japanese male. Analysis of the peripheral blood revealed pancytopenia, and the bone marrow findings showed marked ringed sideroblasts and chromosomal deletion (46XY, l l q -) . 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 eiectrophoresis, ADA lsozyme 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 reticuiocyte mRNA showed no increase In the amount of ADA mRNA in the patient's reticulocytes compared with those of reticuiocyte-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.