Tomonaga M, Jinnai I, Tagawa M, Amenomori T, Nishino K, Yao E, Nonaka H, Kuriyama K, Yoshida Y, Matsuo T
Blood. 1987 Feb;69(2):546-52.
The bone marrow of a patient with acute undifferentiated leukemia developed unique colonies after a 14-day culture in erythropoietin (EPO)-containing methylcellulose. The colonies consisted of 20 to 200 nonhemoglobinized large blast cells. Cytogenetic analysis of single colonies revealed hypotetraploid karyotypes with several marker chromosomes that were identical to those found in directly sampled bone marrow. The concurrently formed erythroid bursts showed only normal karyotypes. No leukemic colony formation was observed in other culture systems with either colony-stimulating activity (CSA) or phytohemagglutinin-stimulated leukocyte-conditioned medium (PHA-LCM). The leukemic colonies exhibited a complete EPO-dose dependency similar to that of the patient's normal BFU-E. Although cytochemical and immunologic marker studies of the bone marrow cells failed to clarify the cell lineage of the leukemic cells with extraordinarily large cell size, ultrastructural study revealed erythroid differentiation such as siderosome formation in the cytoplasm and ferritin particles in the rhophecytosis invaginations. These findings indicate that the patient had poorly differentiated erythroid leukemia and that some of the clonogenic cells might respond to EPO in vitro. Corresponding to this biological feature, the leukemic cells were markedly decreased in number in response to repeated RBC transfusions, and partial remission was obtained. These observations suggest that erythroid leukemia distinct from erythroleukemia (M6) with a myeloblastic component, can develop as a minor entity of human acute leukemia.
一名急性未分化白血病患者的骨髓在含促红细胞生成素(EPO)的甲基纤维素中培养14天后形成了独特的集落。这些集落由20至200个未血红蛋白化的大原始细胞组成。对单个集落的细胞遗传学分析显示为亚四倍体核型,伴有几条标记染色体,与直接采集的骨髓中发现的染色体相同。同时形成的红系爆式集落仅显示正常核型。在其他具有集落刺激活性(CSA)或植物血凝素刺激的白细胞条件培养基(PHA-LCM)的培养系统中未观察到白血病集落形成。白血病集落表现出与患者正常BFU-E相似的完全EPO剂量依赖性。尽管对骨髓细胞进行的细胞化学和免疫标记研究未能明确细胞体积异常大的白血病细胞的细胞系,但超微结构研究显示出红系分化,如细胞质中形成含铁小体以及吞噬小泡内出现铁蛋白颗粒。这些发现表明该患者患有低分化红系白血病,并且一些克隆形成细胞可能在体外对EPO有反应。与此生物学特征相对应,白血病细胞数量因反复输注红细胞而明显减少,并获得了部分缓解。这些观察结果提示,与具有髓母细胞成分的红白血病(M6)不同的红系白血病可作为人类急性白血病的一种少见类型出现。