Clinicopathological study of Japanese patients with genetic iron overload syndromes.
Clinicopathological study of Japanese patients with genetic iron overload syndromes.
复制标题
日本遗传性铁超载综合征患者的临床病理学研究。
DOI：
10.1111/pin.12043
复制
发表时间：
2012
期刊：
影响因子：
--
通讯作者：
A. Hattori
中科院分区：
文献类型：
--
作者：
Sakata N;Takeshita M;Morishuge N;Tashiro T;A. Hattori
急性巨核细胞白血病是一种罕见的疾病，预后差。组织学上，AMGL病例显示不同程度的纤维化和均匀原始细胞或成熟发育不良的巨核细胞的存在。我们检查了18例AMGL，包括特发性（n= 9）和继发性（n= 9）病例。14例为男性，4例为女性，年龄范围为14至87岁（中位数为58岁）。所有病例均有贫血，但白细胞和血小板计数不同。14例患者外周血中检测到原始细胞。16例中有14例染色体异常。中位生存期为6个月（范围，1-48个月）。生存率与纤维化的严重程度、原始细胞的比例和AMGL的病因无关。11例组织化学检查的病例中有9例为血小板衍生生长因子（PDGF）（-BB）阳性，尤其是巨核细胞和少数成纤维细胞。PDGF阳性病例显示不同程度的纤维化，而阴性病例显示无纤维化证据。我们的研究结果证实了AMGL患者的预后不良，无论纤维化的程度，并证明PDGF可以在骨髓纤维化的发病机制中发挥重要作用。
Acute megakaryoblastic leukaemia (AMGL) is an uncommon disease with poor prognosis. Histopathologically, AMGL cases show variable degree of fibrosis and the presence of uniform blasts or mature dysplastic megakaryocytes. Here we examined 18 cases of AMGL, including idiopathic (n= 9) and secondary (n= 9) cases. Fourteen cases were males and four were females, ranging in age from 14 to 87 years (median, 58). All cases had anaemia, but leukocyte and platelet counts varied. Blast cells were detected in the peripheral blood of 14 cases. Fourteen of 16 cases showed chromosomal abnormalities. The median survival was 6 months (range, 1–48 months). Survival rates did not correlate with the severity of fibrosis, proportion of blast cells and cause of AMGL. Nine of the 11 cases examined immunohistochemically were positive for platelet‐derived growth factor (PDGF)(‐BB), especially megakaryoblasts and a few fibroblasts. The PDGF‐positive cases showed various degrees of fibrosis, while the negative cases showed no evidence of fibrosis. Our results confirmed the poor prognosis of patients with AMGL, irrespective of the degrees of fibrosis, and demonstrated that PDGF could play an important role in the pathogenesis of marrow fibrosis.