Lewis S, Oscier D, Boultwood J, Ross F, Fitchett M, Rack K, Abrahamson G, Buckle V, Wainscoat J S
Haematology Department, John Radcliffe Hospital, Oxford, United Kingdom.
Am J Hematol. 1995 Jul;49(3):194-200. doi: 10.1002/ajh.2830490304.
The hematological and clinical features of 26 patients with myelodysplasia and a chromosome 5q deletion in the bone marrow are presented. We have examined the relationship of French-American-British Co-operative Group (FAB) 1982 classification and bone marrow karyotype at diagnosis with patient outcome and the presence or absence of the classical features of the 5q-syndrome. Those patients classified as refractory anemia (RA) with no additional karyotypic abnormalities have the typical features of the 5q-syndrome and a good prognosis. None of the patients in this group transformed to acute leukemia during the period of follow-up. Patients with either refractory anemia and excess blasts (RAEB) or additional karyotypic abnormalities show many of the hematologic features of the 5q-syndrome but do not share the good prognosis. We conclude that the 5q-syndrome may be best defined as primary MDS of the FAB type RA with a 5q deletion as the sole karyotypic abnormality. This simple definition will distinguish patients with a good prognosis and all the classical features of the 5q-syndrome.
本文报告了26例骨髓发育异常且骨髓存在5号染色体长臂缺失患者的血液学及临床特征。我们研究了1982年法美英协作组（FAB）分类、诊断时的骨髓核型与患者预后以及5q-综合征典型特征的有无之间的关系。那些被归类为无其他核型异常的难治性贫血（RA）患者具有5q-综合征的典型特征且预后良好。该组患者在随访期间均未转化为急性白血病。伴有原始细胞过多的难治性贫血（RAEB）患者或有其他核型异常的患者表现出许多5q-综合征的血液学特征，但预后不佳。我们得出结论，5q-综合征最好定义为FAB分型为RA的原发性骨髓增生异常综合征，其唯一的核型异常为5号染色体长臂缺失。这一简单定义将区分出预后良好且具有5q-综合征所有典型特征的患者。
