Riccardi A, Giordano M, Girino M, Cazzola M, Montecucco C M, Cassano E, Danova M, Ucci G, Castello A, Coci A
Blut. 1987 Mar;54(3):153-63. doi: 10.1007/BF00320369.
One hundred and one patients with refractory cytopenia were reviewed for morphological classification (using bone marrow, BM, imprints for cytology and Jamshidi biopsies for BM cellularity) and clinical course. Final diagnoses were: moderate aplastic anemia (MAA), myelodysplastic syndromes (MDS) and hypoplastic acute leukemia (HAL). Ninety-two patients received high dose testosterone enanthate (TE) as first treatment (starting dose = 7-10 mg/week i.m. for at least three months). Median survival was significantly longer in MAA than in MDS and in HAL. Among MDS patients, those with primary acquired sideroblastic (AISA) and refractory (RA) anemia had median survival similar to those with MAA, but distinctly longer (p = 0.01) than patients with RA with an excess of blasts (RAEB), RAEB in transformation (RAEBtr) and chronic myelomonocytic leukemia (CMMoL). Acute leukemia (AL) developed more rarely (p less than 0.02) in MAA, AISA and RA than in RAEB, RAEBtr and CMMoL. Response to TE was seen in about two thirds of MAA and in a half of MDS and HAL patients. Among MDS patients, those with hypocellular BM developed leukemia less frequently, responded to androgens more often and survived longer than those with normocellular and, especially, with hypercellular BM. These data indicate that the cytohistological classification of refractory cytopenias identifies essentially two groups with different clinical behaviour, one (MAA, AISA and RA) having long life expectancy and a low probability of developing AL and the other (RAEB, RAEBtr, CMMoL) with a short survival and relatively frequent leukemic complication. Bone marrow hypocellularity seems to be a favourable prognostic factor in MDS. Patients with refractory cytopenias, especially those with a hypocellular BM, can be advantageously treated with androgens.
对101例难治性血细胞减少症患者进行了形态学分类（采用骨髓、骨髓印片进行细胞学检查以及Jamshidi活检评估骨髓细胞密度）和临床病程回顾。最终诊断为：中度再生障碍性贫血（MAA）、骨髓增生异常综合征（MDS）和低增生性急性白血病（HAL）。92例患者接受大剂量庚酸睾酮（TE）作为初始治疗（起始剂量=7-10mg/周，肌肉注射，至少三个月）。MAA患者的中位生存期显著长于MDS和HAL患者。在MDS患者中，原发性获得性铁粒幼细胞贫血（AISA）和难治性贫血（RA）患者的中位生存期与MAA患者相似，但明显长于伴有过多原始细胞的RA（RAEB）、转化中的RAEB（RAEBtr）和慢性粒-单核细胞白血病（CMMoL）患者（p=0.01）。与RAEB、RAEBtr和CMMoL相比，MAA、AISA和RA中急性白血病（AL）的发生更为罕见（p<0.02）。约三分之二的MAA患者以及一半的MDS和HAL患者对TE有反应。在MDS患者中，骨髓细胞减少的患者发生白血病的频率较低，对雄激素的反应更频繁，生存期也比骨髓细胞正常尤其是骨髓细胞增多的患者更长。这些数据表明，难治性血细胞减少症的细胞组织学分类基本可识别出两组具有不同临床行为的患者，一组（MAA、AISA和RA）预期寿命长，发生AL的可能性低，另一组（RAEB、RAEBtr、CMMoL）生存期短且白血病并发症相对频繁。骨髓细胞减少似乎是MDS的一个有利预后因素。难治性血细胞减少症患者，尤其是骨髓细胞减少的患者，使用雄激素治疗可能有益。
