Mongkonsritragoon W, Letendre L, Li C Y
Division of Hematopathology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Am J Hematol. 1998 Nov;59(3):252-7. doi: 10.1002/(sici)1096-8652(199811)59:3<252::aid-ajh14>3.0.co;2-c.
Benign nodular lymphoid lesions are not rare in the bone marrow of patients with myelodysplastic syndrome (MDS). Herein, we report a case of MDS with clonal lymphoid aggregates in the bone marrow but without evidence of systemic lymphoma. The case of a 71-year-old man was evaluated for cytopenia. His bone marrow was initially hypocellular, with 10% blasts and a few small lymphoid aggregates. The diagnosis of refractory anemia with excess blasts was made. The disease progressed gradually, and he received erythropoietin and granulocyte colony-stimulating factor for a short time. Forty-two months later, acute leukemia (M1) developed, with 60% to 70% blasts in the bone marrow. The bone marrow also showed large aggregates of lymphocytes. Immunohistochemical study of these cells in the nodular lesions showed 50% CD3+ and 50% CD20+. Cytogenetic and molecular genetic studies revealed monosomy 7 and T- and B-cell clonal gene rearrangement. Fluorescent in situ hybridization study with centromere-specific probes of a bone marrow specimen showed monosomy 7 in both nodular lymphoid lesions and surrounding bone marrow cells, indicating that both processes originated from the same abnormal pluripotential progenitor.
良性结节性淋巴样病变在骨髓增生异常综合征(MDS)患者的骨髓中并不罕见。在此,我们报告一例骨髓中有克隆性淋巴样聚集但无系统性淋巴瘤证据的MDS病例。一名71岁男性因血细胞减少接受评估。其骨髓最初细胞减少,原始细胞占10%,并有一些小的淋巴样聚集。诊断为伴有过多原始细胞的难治性贫血。疾病逐渐进展,他短期接受了促红细胞生成素和粒细胞集落刺激因子治疗。42个月后,发展为急性白血病(M1型),骨髓中原始细胞占60%至70%。骨髓中还可见大量淋巴细胞聚集。对结节性病变中的这些细胞进行免疫组化研究显示,50%为CD3+,50%为CD20+。细胞遗传学和分子遗传学研究发现7号染色体单体以及T细胞和B细胞克隆性基因重排。对一份骨髓标本用着丝粒特异性探针进行荧光原位杂交研究显示,结节性淋巴样病变及周围骨髓细胞均存在7号染色体单体,表明这两个过程均起源于同一个异常的多能祖细胞。