The impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis: a prospective survey of 202 cases in Japan
The impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis: a prospective survey of 202 cases in Japan
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DOI：
10.1111/j.1600-0609.2009.01298.x
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发表时间：
2009-10-01
影响因子：
3.1
通讯作者：
Shimoda, Kazuya
中科院分区：
文献类型：
--
作者：
Hidaka, Tomonori;Shide, Kotaro;Shimoda, Kazuya
细胞遗传学异常常见于原发性骨髓纤维化患者。据报道，存在特定的细胞遗传学异常，如染色体13 q-、20 q-或-7/7 q-的唯一异常，对原发性骨髓纤维化的预后有影响。我们分析了1999年以来日本原发性骨髓纤维化患者前瞻性调查的数据，以阐明细胞遗传学异常对原发性骨髓纤维化预后的影响。共有202例原发性骨髓纤维化患者有细胞遗传学和预后数据。202例病例中有80例（40%）有细胞遗传学异常，血小板计数明显相关。虽然异常核型的存在并不影响预后，但与正常核型或仅有13 q或20 q异常的患者相比，13 q和20 q以外的细胞遗传学异常的原发性骨髓纤维化患者的预后较差。与细胞遗传学特征良好的患者（细胞遗传学正常，染色体13 q-或20 q-的唯一异常）相比，细胞遗传学特征不良的患者（13 q-或20 q-以外的异常细胞遗传学）转化为白血病的趋势更大。在原发性骨髓纤维化患者中，13 q或20 q以外的异常细胞遗传学对生存率和白血病转化风险的预后影响均较差。
Cytogenetic abnormalities were often observed in primary myelofibrosis patients. The presence of specific cytogenetic abnormalities, such as sole abnormalities of chromosome 13q-, 20q-, or -7/7q-, is reported to have the influence on the prognosis of primary myelofibrosis. We analyzed the data from the prospective survey of Japanese primary myelofibrosis patients which was conducted from 1999 to clarify the impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis. A total of 202 primary myelofibrosis patients had the cytogenetic and the prognostic data. Eighty (40%) out of 202 cases had cytogenetic abnormalities, and an association was evident for platelet counts. Although the presence of an abnormal karyotype did not affect the prognosis, primary myelofibrosis patients with cytogenetic abnormalities other than 13q- and 20q- showed an inferior prognosis compared to patients with a normal karyotype or sole 13q- or 20q- abnormalities. Patients with an unfavorable cytogenetic profile (abnormal cytogenetics other than 13q- or 20q-) also had a greater tendency to transform to leukemia than patients with a favorable cytogenetic profile (normal cytogenetics, sole abnormalities of either chromosome 13q-, or 20q-). Abnormal cytogenetics other than 13q- or 20q- in primary myelofibrosis patients has the poor prognostic effect for both survival and the risk of leukemic transformation.