Lambertenghi-Deliliers G, Annaloro C, Oriani A, Soligo D, Pozzoli E, Polli E E
Istituto di Scienze Mediche, University of Milan, Fondazione Matarelli, Italy.
Ann Hematol. 1993 Feb;66(2):85-91. doi: 10.1007/BF01695890.
Bone marrow biopsy (BMB) has aroused growing interest as a possible aid in the diagnostic and prognostic evaluation of myelodysplastic syndromes (MDS). Previous reports have pointed out that MDS patients with blastic aggregates or severe bone marrow (BM) fibrosis are characterized by a worse clinical outcome. BMBs of 106 MDS patients were retrospectively reviewed, and relationships among the different histological parameters as well as clinicopathological correlations were looked for. Three patterns of BM blastic infiltration ("diffuse," "cluster," and "large") were recognized. Overt leukemic transformation and overall survival were selected as prognostic end points. BM infiltration was "diffuse" in 18, "cluster" in 48, and "large" in 40 cases. RAEB-t patients accounted for about half of the "large" cases, and none had a "diffuse" pattern (p < 0.01). Nineteen patients showed extensive BM fibrosis; most of them were characterized by "cluster" blastic infiltration and megakaryocyte hyperplasia. Leukemic transformation occurred in 67% of "large" cases (p< 0.001) and in none of the "cluster" cases with severe BM fibrosis (p < 0.01); however, survival was equally poor in these two groups because of early leukemic transformation (large cases) and BM failure (cluster cases). The FAB classification did not significantly correlate with prognosis. Patients with "cluster" BM infiltration and severe fibrosis can be regarded as a true separate MDS subset characterized by unique clinicopathological and prognostic features. Because of the subacute clinical behavior of most cases, and the poor performance status of many elderly patients, there is still controversy as to the best therapeutic approach in MDS. Histological analysis allowed two groups of MDS patients to be identified, both characterized by poor life expectancy, who could benefit from early aggressive chemotherapy.
骨髓活检（BMB）作为骨髓增生异常综合征（MDS）诊断和预后评估的一种可能辅助手段，已引起越来越多的关注。既往报道指出，伴有原始细胞聚集或严重骨髓（BM）纤维化的MDS患者临床结局较差。对106例MDS患者的骨髓活检进行回顾性分析，探寻不同组织学参数之间的关系以及临床病理相关性。识别出三种骨髓原始细胞浸润模式（“弥漫性”、“簇状”和“大片状”）。选择明显白血病转化和总生存作为预后终点。18例骨髓浸润为“弥漫性”，48例为“簇状”，40例为“大片状”。难治性贫血伴原始细胞增多-转变型（RAEB-t）患者约占“大片状”病例的一半，且无一例为“弥漫性”模式（p<0.01）。19例患者表现为广泛的骨髓纤维化；其中大多数以“簇状”原始细胞浸润和巨核细胞增生为特征。67%的“大片状”病例发生白血病转化（p<0.001），而严重骨髓纤维化的“簇状”病例无一发生白血病转化（p<0.01）；然而，由于早期白血病转化（大片状病例）和骨髓衰竭（簇状病例），这两组患者的生存同样较差。FAB分类与预后无显著相关性。伴有“簇状”骨髓浸润和严重纤维化的患者可被视为一个真正独立的MDS亚组，具有独特的临床病理和预后特征。由于大多数病例的临床行为呈亚急性，且许多老年患者的体能状态较差，MDS的最佳治疗方法仍存在争议。组织学分析可识别出两组预期寿命均较短的MDS患者，他们可能从早期积极化疗中获益。
