Tamai Y, Takami H, Nakahata R, Nakui Y, Suzuki Y, Akagi T, Kariya K, Tsushima K, Munakata A, Kawamura S
First Department of Internal Medicine, Hirosaki University School of Medicine.
Rinsho Ketsueki. 1997 Dec;38(12):1243-8.
During 1990 to 1993, five patients who had been treated in our hospital for aplastic anemia (AA) with a normal karyotype, were found to have hypoplastic myelodysplastic syndromes (hMDS); three patients with RA, one RAEB, and one RAEB-T, and all of them have chromosomal abnormalities. The mean age of the hMDS patients (4 men, 1 woman) was 51.0 years (range 31-61). We retrospectively studied the hematological features of the 5 cases in the early stage without chromosomal abnormalities. They showed, 1) appearance of erythroblasts (3/5), 2) more reticulocyte counts than typical AA (mean 7.8 x 10(4)/microliter), 3) macrocytic anemia (mean MCV 106fl) in peripheral blood, 4) relative erythroid hyperplasia (M/E ratio 0.2-1.5), and 5) low lymphocyte counts (mean percentage of lymphocytes 32, 8%) in bone marrow. Considering about the differences or transformation between AA and hMDS, it is interesting that all five patients have initially had a normal karyotype and subsequently developed chromosomal abnormalities.
1990年至1993年期间，我院收治的5例核型正常的再生障碍性贫血（AA）患者被发现患有低增生性骨髓增生异常综合征（hMDS）；其中3例为难治性贫血（RA），1例为环形铁粒幼细胞难治性贫血（RAEB），1例为转化型难治性贫血伴原始细胞增多（RAEB-T），且所有患者均有染色体异常。hMDS患者（4男1女）的平均年龄为51.0岁（范围31 - 61岁）。我们回顾性研究了这5例早期无染色体异常患者的血液学特征。结果显示，1）出现幼红细胞（3/5），2）网织红细胞计数高于典型AA（平均7.8×10⁴/微升），3）外周血大细胞性贫血（平均MCV 106fl），4）相对红系增生（M/E比值0.2 - 1.5），5）骨髓淋巴细胞计数低（淋巴细胞平均百分比32.8%）。考虑到AA与hMDS之间的差异或转化，有趣的是，所有5例患者最初核型均正常，随后出现染色体异常。
