Arathi C A, Puttaraj K R, Shobha S N
Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India.
J Lab Physicians. 2011 Jan;3(1):49-51. doi: 10.4103/0974-2727.78568.
Myelodysplastic syndrome (MDS) represents one of the most challenging health-related problems in the elderly, characterized by dysplastic morphology in the bone marrow in association with ineffective hematopoiesis. Hypoplastic MDS (h-MDS) accounts for 12-17% of all patients with MDS and has yet to be shown to alter the disease course or prognosis. The concept that T-cell-mediated autoimmunity contributes to bone marrow failure in MDS has been widely accepted due to hematologic improvement after immunosuppressive therapy. T-cell expansion is known to occur in these patients, but development of chronic T-cell disorders, especially T-prolymphocytic leukemia (PLL) in a hypocellular MDS is extremely rare, which has an aggressive course. The possible explanation for the association between the two disorders is that T-PLL might arise from a clonally arranged MDS stem cell. We report a unique case of h-MDS with non-progressive pancytopenia and severe hypocellular marrow for 2 years, followed by T-PLL within few months.
骨髓增生异常综合征（MDS）是老年人面临的最具挑战性的健康相关问题之一，其特征为骨髓发育异常形态并伴有无效造血。低增生性MDS（h-MDS）占所有MDS患者的12% - 17%，尚未显示会改变疾病进程或预后。由于免疫抑制治疗后血液学改善，T细胞介导的自身免疫导致MDS骨髓衰竭这一概念已被广泛接受。已知这些患者会出现T细胞扩增，但慢性T细胞疾病的发生，尤其是低细胞性MDS中的T - 前淋巴细胞白血病（PLL）极为罕见，其病程侵袭性强。这两种疾病关联的可能解释是T - PLL可能源自克隆性排列的MDS干细胞。我们报告了一例独特的h-MDS病例，患者有两年的非进行性全血细胞减少和严重低细胞骨髓，随后在几个月内发展为T - PLL。
