Tomonaga M, Jinnai I, Kuriyama K, Nonaka H, Amenomori T, Matsuo T, Yoshida Y, Sasagawa I, Sadamori N, Ichimaru M
Rinsho Ketsueki. 1989 Oct;30(10):1788-99.
A series of 116 patients with MDS consisted of 74 cases of RA, 10 cases of RARS, 14 cases of RAEB, 9 cases of RAEB-T and 9 cases of CMML, were studied on the quantity and morphological abnormalities of megakaryocytes in relation to over all survival and leukemic change. The amount of megakaryocytes was graded into four groups; marked hypoplasia (O), moderate hypoplasia (L), normoplasia (N) and hyperplasia (H), RA cases showed heterogeneous pattern; containing 14 cases (18.9%) of group (O), 18 cases (24.3%) of group (L), 31 cases (41.9%) of group (N) and 11 cases (14.9%) of group (H). RARS, RAEB, RAEB-T and CMML cases were classified into group (N) or group (H). The heterogeneous pattern of RA did not relate to leukemic change, but over all survival tended to be shorter in group (N) cases. A significant number of young female cases of RA were involved in group (O). Morphological abnormalities of MDS megakaryocytes were classified into five types; I, mononuclear micromegakaryocytes, II, binuclear micromegakaryocytes, III, mononuclear small megakaryocytes, IV, multiseparated-nuclear megakaryocytes and V, megakaryocytes with bizzare nuclei. RAEB and RAEB-T cases uniformly showed marked dysmegakaryopoiesis ranging from type I to V. whereas RA, RARS and CMML cases showed mild dysmegakaryopoiesis. Only five cases (6.4%) of RA cases had type I micromegakaryocytes. Eight RA cases with type I on diagnosis or obtaining it during the clinical course tended to develop acute myeloid leukemia (5 cases) or to transform to RAEB sooner or later. In two cases of RAEB in which hematological improvement was obtained with low dose cytosine arabinoside regimen, disappearance of type I micromegakaryocytes was noted. A female case with 5q-anomaly surviving more than 10 years showed marked megakaryocyte hyperplasia and almost exclusively type III and IV megakaryocytes. These findings indicated that pattern of dysmegakaryopoiesis, especially appearance of type I, was closely related to leukemic change in MDS. Thus quantitative and qualitative evaluations of MDS megakaryocytopoiesis seemed important to understand the further heterogeneity of pathophysiology in MDS subtypes.
对116例骨髓增生异常综合征（MDS）患者进行了研究，其中包括74例难治性贫血（RA）、10例环形铁粒幼细胞性难治性贫血（RARS）、14例难治性贫血伴原始细胞增多（RAEB）、9例难治性贫血伴原始细胞增多-转变型（RAEB-T）和9例慢性粒-单核细胞白血病（CMML），研究了巨核细胞的数量和形态异常与总体生存及白血病转化的关系。巨核细胞数量分为四组：显著发育不全（O）、中度发育不全（L）、正常发育（N）和增生（H）。RA病例呈现异质性模式；包括O组14例（18.9%）、L组18例（24.3%）、N组31例（41.9%）和H组11例（14.9%）。RARS、RAEB、RAEB-T和CMML病例分为N组或H组。RA的异质性模式与白血病转化无关，但N组病例的总体生存往往较短。大量年轻女性RA病例属于O组。MDS巨核细胞的形态异常分为五种类型：I型，单核微巨核细胞；II型，双核微巨核细胞；III型，单核小巨核细胞；IV型，多分叶核巨核细胞；V型，核异形巨核细胞。RAEB和RAEB-T病例均显示从I型到V型的明显巨核细胞生成异常，而RA、RARS和CMML病例显示轻度巨核细胞生成异常。仅5例（6.4%）RA病例有I型微巨核细胞。8例诊断时或临床过程中出现I型的RA病例倾向于发展为急性髓系白血病（5例）或迟早转化为RAEB。在2例采用低剂量阿糖胞苷方案获得血液学改善的RAEB病例中，I型微巨核细胞消失。1例存活超过10年的5q-异常女性病例显示巨核细胞显著增生，几乎全是III型和IV型巨核细胞。这些发现表明，巨核细胞生成异常模式，尤其是I型的出现，与MDS中的白血病转化密切相关。因此，对MDS巨核细胞生成进行定量和定性评估似乎对于理解MDS亚型病理生理学的进一步异质性很重要。
