Goldberg S L, Noel P, Klumpp T R, Dewald G W
Division of Hematology, Mayo Clinic and Foundation Rochester, Minnesota, USA.
Am J Clin Oncol. 1998 Feb;21(1):42-7. doi: 10.1097/00000421-199802000-00010.
Pure erythroid malignancies, such as Di Guglielmo disease (DG), in which the predominant immature elements are proerythroblasts, are excluded from the French-American-British (FAB) classification for acute leukemia and do not fit neatly into any of the categories of myelodysplasia. This retrospective review compares the clinical and laboratory features of DG and erythroleukemia (FAB M6) among 37 cases treated at a single institution over a 7-year period. DG was defined as >30% proerythroblasts and the absence of a myeloblastic component. Clinical and laboratory features were similar in both subtypes. High proportions of secondary leukemias and prior myelodysplastic syndromes (MDS) were noted (M6, 13 of 26 cases; DG, five of 11 cases; p = 0.85). Pancytopenia was common at presentation in both groups [median white blood cells (WBC), 2,600/mm3; HgB, 8.65 gm/dl; platelets, 38,000/microl]. Two-thirds of studied cases had chromosomal abnormalities typified by major karyotypic abnormalities (MAKA) involving three or more chromosomes. Abnormalities involving chromosome 5 and/or 7 occurred in 47% (48% M6 and 45% DG). Both erythroid malignancies carried a poor prognosis (M6, 6.0-month median survival; DG, 4-month survival; p = 0.74). Among those patients choosing aggressive rather than palliative therapy, higher remission rate (80 versus 25%) and survival advantage (11.5 versus 2.5 months) were seen in M6 compared to DG. However, only two long-term survivors exist. The similar clinical and laboratory features, cytogenetic patterns, and poor survival data suggest that the FAB classification schema should be modified to include DG.
纯红系恶性肿瘤，如迪古列尔莫病（DG），其主要的未成熟细胞成分为早幼红细胞，被排除在急性白血病的法美英（FAB）分类之外，也不完全符合骨髓增生异常综合征的任何类别。这项回顾性研究比较了在一家机构7年期间治疗的37例患者中DG和红白血病（FAB M6）的临床和实验室特征。DG定义为早幼红细胞>30%且无髓母细胞成分。两种亚型的临床和实验室特征相似。观察到继发性白血病和既往骨髓增生异常综合征（MDS）的比例较高（M6，26例中的13例；DG，11例中的5例；p = 0.85）。两组患者就诊时全血细胞减少很常见[白细胞（WBC）中位数，2600/mm³；血红蛋白（HgB），8.65 g/dl；血小板，38000/微升]。三分之二的研究病例有以涉及三条或更多染色体的主要核型异常（MAKA）为特征的染色体异常。涉及5号和/或7号染色体的异常在47%的病例中出现（M6为48%，DG为45%）。两种红系恶性肿瘤的预后都很差（M6，中位生存期6.0个月；DG，生存期4个月；p = 0.74）。在选择积极治疗而非姑息治疗的患者中，与DG相比，M6的缓解率更高（80%对25%）且生存优势更明显（分别为11.5个月和2.5个月）。然而，仅有两名长期存活者。相似的临床和实验室特征、细胞遗传学模式以及较差的生存数据表明FAB分类方案应进行修改以纳入DG。
