Maschek H, Kaloutsi V, Werner M, Bandecar K, Buhr T, Georgii A
Pathologisches Institut, Medizinischen Hochschule Hannover.
Verh Dtsch Ges Pathol. 1990;74:144-8.
In a retrospective study, 45 (19.4%) out of 232 patients with MDS revealed myelosclerosis (MS) in bone marrow biopsy (BMB). Histological classification according to FAB criteria showed the following distribution: RA 21 (47%), RARS 1 (2%), RAEB 9 (20%), RAEB-T 3 (7%), and CMMol 11 (24%). Sclerosis occurred in all subtypes of MDS, but with a higher incidence in CMMol. Clinical data showed lower values of hemoglobin and lower platelet counts in MDS.MS. Life expectancy was reduced to 7.8 months, compared with 15.0 months in MDS without MS (p = 0.0026). In RA, the survival times were 9.7 months in MDS.MS, compared to 27.9 months in MDS without MS (p = 0.0035). 21 (47%) of the patients with MDS.MS experienced a transformation into ANLL. Myelosclerosis therefore seems to herald a bad prognosis.
在一项回顾性研究中，232例骨髓增生异常综合征（MDS）患者中有45例（19.4%）在骨髓活检（BMB）中显示有骨髓硬化（MS）。根据FAB标准进行的组织学分类显示如下分布：难治性贫血（RA）21例（47%）、难治性贫血伴环形铁粒幼细胞增多（RARS）1例（2%）、难治性贫血伴原始细胞增多（RAEB）9例（20%）、难治性贫血伴原始细胞增多转变型（RAEB-T）3例（7%）、慢性粒-单核细胞白血病（CMMoL）11例（24%）。骨髓硬化发生于MDS的所有亚型，但在CMMoL中发生率更高。临床数据显示，MDS合并MS患者的血红蛋白值和血小板计数较低。预期寿命降至7.8个月，而无MS的MDS患者为15.0个月（p = 0.0026）。在RA中，MDS合并MS患者的生存时间为9.7个月，而无MS的MDS患者为27.9个月（p = 0.0035）。21例（47%）MDS合并MS患者发生了向急性非淋巴细胞白血病（ANLL）的转化。因此，骨髓硬化似乎预示着预后不良。
