Kondo H, Takaso T
Department of Medicine, Shimizu Kousei Hospital.
Rinsho Ketsueki. 1992 Dec;33(12):1851-6.
A 56-year-old man had a leiomyosarcoma of the small intestine in 1987. After surgery, he received cyclophosphamide for 2 years. In December, 1990, he exhibited severe pancytopenia. His hematological data were as follows: Hb 7.4g/dl, ret. 0.8%, WBC 1,700/microliters with leukoerythroblastosis and 2.8 x 10(4)/microliters platelets. A bone marrow aspiration was a dry tap. A bone marrow biopsy specimen showed a hypercellular marrow with myelofibrosis, leukemic infiltration (10.2%) and slight dyserythropoiesis. Both PPO and GPIIb/IIIa reaction were positive for blast cells and atypical megakaryoblasts. A diagnosis of MDS with an abnormality in megakaryocytic lineage was made. The patient was treated with 1,25-dihydroxy-vitamin D3, however this therapy was temporary and he developed into acute megakaryoblastic leukemia (M7). This report suggested that some cases of therapy-related leukemia (TRL) mainly involve megakaryocytic lineage and are diagnosed as MDS with myelofibrosis which transform to M7. The fact that PAS stain of erythroblasts in the patient reported here was positive may suggest involvement of development of more precise immunological markers of differentiation and EM study will permit better diagnosis of TRL and may therefore facilitate new therapeutic approaches.
一名56岁男性在1987年患小肠平滑肌肉瘤。手术后，他接受了2年的环磷酰胺治疗。1990年12月，他出现严重全血细胞减少。其血液学数据如下：血红蛋白7.4g/dl，网织红细胞0.8%，白细胞1700/微升，伴有幼粒-幼红细胞血象，血小板2.8×10⁴/微升。骨髓穿刺为干抽。骨髓活检标本显示骨髓细胞增多伴骨髓纤维化、白血病浸润（10.2%）和轻度红细胞生成异常。原始细胞和非典型巨核母细胞的PPO和GPIIb/IIIa反应均为阳性。诊断为巨核细胞系异常的骨髓增生异常综合征。该患者接受了1,25-二羟基维生素D3治疗，但该治疗是暂时的，他发展为急性巨核细胞白血病（M7）。本报告提示，一些治疗相关白血病（TRL）病例主要累及巨核细胞系，被诊断为伴有骨髓纤维化的骨髓增生异常综合征，后者可转化为M7。本文报道患者的幼红细胞PAS染色阳性这一事实可能提示参与了更精确的分化免疫标志物的发育，而电镜研究将有助于更好地诊断TRL，因此可能促进新的治疗方法。
