摘要
目的探讨低增生骨髓增生异常综合征（ＭＤＳ）的由来和发展。方法对我院１０年中确诊的２５例低增生ＭＤＳ进行了系统分析，对其中１７例患者进行长期追访。结果（１）低增生ＭＤＳ占同期确诊为ＭＤＳ的２１９例患者中１１．４％，确诊时平均年龄为（４４．８±１４．７）岁。（２）ＦＡＢ分型：难治性贫血（ＲＡ）１１例，难治性贫血伴原始细胞增多（ＲＡＥＢ）１４例。（３）低增生ＭＤＳ很可能是ＭＤＳ患者病程中一个阶段，其骨髓增生活跃和低下可以相互转化，这种转化不但可以发生在同一ＦＡＢ亚型内，也可以发生在不同亚型相互转化时。（４）长期随访的１７例患者中有７例转为急性白血病，占４１．２％，６例为急性粒细胞白血病，１例为急性淋巴细胞白血病；７例中３例转为低增生白血病，４例为增生活跃或极度活跃白血病。（５）１７例患者中７例自低增生ＲＡＥＢ转为急性白血病时间为１～７４个月，中数为２７个月。（６）低增生ＭＤＳ的产生与治疗药物无明显相关。结论低增生ＭＤＳ很可能为ＭＤＳ病程中一个阶段而非一种特殊类型。
Objective To study the origin and progress of myelodysplastic syndrome (MDS) with
hypoplasia. Methods The data of twentyfive cases of hypomyeloplastic MDS diagnosed by our
department in the last ten years were analyzed . 17 of the 25 cases were followed up for a long
time. Results (1) The percentage of hypomyeloplastic MDS was 11.4% of the total 219 MDS
patients. The median age of the 25 cases was (44.814.7) years. (2) FAB subtype: There were 11
cases of RA and 14 of RAEB. (3) Hypomyeloplasic MDS seems to be a developmental phase in
the clinical course in some of the patients and not a special type of MDS. Hyper- and
hypo-myeloplasia could be transformed from one to another . The transformation of
myeloplasia could occur either in the same or and different FAB subtype. (4)Seven of the
seventeen cases transformed to acute leukemia (41.2%), 6 cases were AML and 1 was ALL.3 of
the 7 cases transformed to hypomyeloplastic leukemia and the remaining 4 transformed to
hypermyeloplastic leukemia. (5) The median time from the diagnosis of RAEB to leukemia
transformation , was 27 months in 7 cases with hypoplastic RAEB. (6) No relationship was
found between therapeutic medicines and development of hypomyeloplastic MDS. Conclusion
It is suggested that hypomyeloplastic MDS is probably a developmental phase in the clinical
course of MDS , but not a special type.
出处
《中华内科杂志》
CAS
CSCD
北大核心
1999年第6期370-372,共3页
Chinese Journal of Internal Medicine
