Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd., Unit 72, Houston, TX 77030, USA.
Ann Hematol. 2013 Oct;92(10):1335-43. doi: 10.1007/s00277-013-1776-3. Epub 2013 May 10.
The presence of moderate to severe bone marrow (BM) fibrosis has been shown to be an adverse feature in patients with primary myelodysplastic syndromes (MDS). However, the clinical importance of BM fibrosis is not clear in therapy-related MDS. We retrieved all therapy-related MDS (t-MDS) cases (n = 266) diagnosed at our hospital over a 10-year period (2003-2012). Reticulin and trichrome stains were performed in cases in which BM fibrosis was suspected on initial evaluation of hematoxylin and eosin-stained slide. BM fibrosis was graded according to European consensus guidelines, and a score of MF2/MF3 was defined as moderate/severe fibrosis. Moderate/severe BM fibrosis was found in 47 (17%) patients. Compared to 219 patients with no/mild BM fibrosis, the patients with moderate/severe fibrosis presented with severer thrombocytopenia (p = 0.039) and higher numbers of circulating blasts (p = 0.051) but with similar degrees of anemia and neutropenia, transfusion requirements, and similar incidences of hepatosplenomegaly and constitutional symptoms. Histological examination revealed a comparable BM cellularity and BM blast percentage, but markedly increased megakaryocytes (p < 0.001) in the fibrotic group. Although the risk distribution of cytogenetic data was similar according to the New Comprehensive Cytogenetic Scoring criteria, -5 and -17 were more frequently observed in t-MDS with moderate/severe BM fibrosis (p = 0.031 and p = 0.043, respectively). With a median follow-up of 11.5 months, patients with moderate/severe BM fibrosis showed a similar risk of acute myeloid leukemia transformation and a comparable overall survival in univariate and multivariate analyses. Moderate/severe BM fibrosis in patients with t-MDS is associated with certain clinicopathological and genetic features. However, unlike the situation in patients with primary MDS, moderate/severe BM fibrosis does not add additional risk to patients with therapy-related MDS.
骨髓（BM）纤维化程度为中重度已被证实是原发性骨髓增生异常综合征（MDS）患者的不良特征。然而，在治疗相关性 MDS（t-MDS）中，BM 纤维化的临床重要性尚不清楚。我们检索了我院在 10 年内（2003-2012 年）诊断的所有 t-MDS 病例（n=266）。在苏木精和伊红染色初始评估怀疑 BM 纤维化的病例中进行网状纤维和三色染色。根据欧洲共识指南对 BM 纤维化进行分级，MF2/MF3 评分为中重度纤维化。47（17%）例患者存在中重度 BM 纤维化。与 219 例无/轻度 BM 纤维化患者相比，中重度纤维化患者血小板计数明显降低（p=0.039），循环原始细胞数更高（p=0.051），但贫血和中性粒细胞减少程度、输血需求以及肝脾肿大和全身症状的发生率相似。组织学检查显示，纤维化组的 BM 细胞数和 BM 原始细胞比例相似，但巨核细胞明显增加（p<0.001）。尽管根据新的综合细胞遗传学评分标准，细胞遗传学数据的风险分布相似，但在中重度 BM 纤维化的 t-MDS 中，-5 和 -17 更常见（p=0.031 和 p=0.043）。中位随访 11.5 个月，在单因素和多因素分析中，中重度 BM 纤维化患者的急性髓系白血病转化风险相似，总生存相似。t-MDS 患者中中重度 BM 纤维化与某些临床病理和遗传特征相关。然而，与原发性 MDS 患者的情况不同，中重度 BM 纤维化不会给 t-MDS 患者带来额外的风险。
