Srivastava Swati, Naseem Shano, Gupta Ritu, Kashyap Rajesh, Chaudhary Rajender
Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, 226 014 UP India.
Indian J Hematol Blood Transfus. 2009 Mar;25(1):33-5. doi: 10.1007/s12288-009-0008-4. Epub 2009 Apr 6.
Myelodysplastic syndrome (MDS) comprises a group of stem cell disorders with considerable clinical and morphological heterogeneity. We report a case of MDS in a middle-aged male with clinical features of sepsis, dysplastic neutrophils and 6% blasts on bone marrow aspirate. A clone of neutrophils with deficient expression of CD16, CD55 and CD59 was found. A diagnosis of MDS with excess blasts with co-existent paroxysmal nocturnal hemoglobinuria (PNH) was made. Within a fortnight, the patient progressed to acute myeloid leukemia. We are reporting this unusual case of MDS displaying a sizeable clone of dysplastic neutrophils deficient in glycosyl phosphatidyl inositol anchored proteins, highlighting a common origin of PNH and leukemic clone.
骨髓增生异常综合征（MDS）是一组具有显著临床和形态学异质性的干细胞疾病。我们报告一例中年男性MDS病例，其具有败血症的临床特征、发育异常的中性粒细胞以及骨髓穿刺涂片上6%的原始细胞。发现了一个CD16、CD55和CD59表达缺陷的中性粒细胞克隆。诊断为伴有原始细胞增多的MDS合并阵发性睡眠性血红蛋白尿（PNH）。在两周内，患者进展为急性髓系白血病。我们报告这例不寻常的MDS病例，其显示出一个相当大的糖基磷脂酰肌醇锚定蛋白缺陷的发育异常中性粒细胞克隆，突出了PNH和白血病克隆的共同起源。
