骨髓增生异常综合征的诊断与鉴别诊断─附16例临床病理分析

Bone marrow biopsy pathologic diagnosis of myelodysplastic syndromes and differential diagnosis be-tween hypoplastic myelodysplastic syndromes and chronic aplastic anemia

目的探讨骨髓增生异常综合征的病理诊断标准及其低增生型与慢性再生障碍性贫血的鉴别诊断要点。方法观察１６例骨髓增生异常综合征和１５例慢性再生障碍性贫血骨髓活检塑料包埋切片的病理组织学特点。结果１６例骨髓增生异常综合征中骨髓增生极度活跃、较活跃者各５例（共占６２．５％），增生正常范围者２例（占１２．５％），４例低增生型（占２５％），其增生度在２０％－４０％；所有病例皆可见到三系细胞的发育异常，尤其单圆核巨核细胞较多见；细胞增生越活跃，幼稚细胞越多；幼稚前体细胞异常定位阳性率６８．７５％。其中，４例低增生型骨髓增生异常综合征有２例幼稚前体细胞异常定位阳性，巨核细胞平均数７．６个／ｍｍ２，中幼及以上阶段粒、红细胞平均数１４１．２个／ｍｍ２。１５例慢性再生障碍性贫血中１４例骨髓增生度小于２０％，１例在２０％－４０％；未见细胞形态异常，亦无幼稚前体细胞异常定位阳性病例，不见巨核细胞；中幼及以上阶段粒、红细胞平均数１３．８６个／ｍｍ２，与低增生型骨髓增生异常综合征有极显著差异（Ｐ＜０．０１）；另有２例慢性再生障碍性贫血查见较多浆细胞。

Objective To study the pathologic diagnostic criteria of myelodysplastic syndromes (MDS) and the differential di agnostic criteria between hypoplastic myelopsplastic syndromes (HMDS) and chronic aplastic anenia (CAA). Methods Histopathological methods were used to study the morphological characteristics of 16 cases of MDS and 15 CAA. Results InWDS, an extreme hypercellularity was observed in 5 cases and hypercellularity in 5 cases (62.5% ), a normal cellularity in 2(12.5%), and hypocellularity in 4 (25%), the degree of proliferation varied between 20% and 40%. All three cell lines of MDS showed morphological abnormalities, especially mononuclear megakaryocytes were easily found; the more cellular prolifera-prolifera-tion, the more immature cells; the abnormal localization of immature precursors (ALIP) was postive in 68.25% of MDS; 2 casesof 4 HMDS were ALIP peitive, mean megakaryocyte count of HMDS were 7.6/mm2, the mean immature cells of intermediate andpre-intermediate stages was 141 .2/mm2, the degree of bone marrow proliferation in 14 cases of CAA was < 20%, only in 1 caseit varied between 20% and 40%, all cases of CAA showed no morphological abnormality , no ALIP, no megakaryocyte, and themean immature cells of intermediate and pre-intermediate stages was 13. 86/mm2, significantly lower than that of HMDS (P <0.01). In addition, more plasma cells were found in 2 cases of CAA. Conclusions The diagnosis of MDS and the differential diagnosis between HMDS and CAA should be based on the general analysis of bone marrow cellularity , cell constituents, morpho -logical abnormalities and topographic distortion.