急性颗粒增多的早幼粒细胞白血病细胞形态学分型的探讨(英文)

Inquirement on the Morphological Classification of Acute Hypergranular Promeylocytic Leukemia and Its Clinical Characteristics

目的解决急性早幼粒细胞白血病(M3)目前国内因粗细颗粒混杂而造成的分型困难。方法以粗颗粒型早幼粒细胞的百分率进行分型,规定:粗颗粒型早幼粒细胞>70%为M3a(粗颗粒型);30%<粗颗粒型早幼粒细胞≤70%为M3b(混合颗粒型);粗颗粒型早幼粒细胞≤30%为M3c(细颗粒型)。结果 208例急性早幼粒细胞白血病,其三种亚型的发生率M3a为42.31%,M3b为30.13%,M3c为27.56%;性别分布M3a男性明显多于女性,M3b无性别差异,M3c女性明显多于男性;年龄分布M3a<M3b<M3c;初诊时发热症状M3b最多见,M3a次之,M3c较少见;出血情况M3a最多见,M3b次之,M3c较少见;部分M3a、M3b的病例胸骨压痛明显,M3c未见有之。M3a部分病例有肝肿大,M3b与M3c未见肝肿大者;三型的的部分病例均见有脾肿大;但三者之间无明显差异(P>0.05)。淋巴结肿大以M3b为多,M3a次之,M3c未见肿大者。Hb、RBC、BPC、WBC的均值M3a<M3b<M3c;外周血中早幼粒细胞的数值M3c>M3a>M3b;骨髓有核细胞的增生M3a多为增生极度活跃,M3b、M3c多为增生明显活跃;粒红比值、早幼粒细胞的百分率、粗颗粒型早幼粒细胞的比值均M3a>M3b>M3c;Auer小体的发生M3b多见,M3c较少见,M3a不易见。结论三种亚型不论在性别、年龄分布上,还是在主要临床症状、血象、骨髓象上均有不同的表现与特征。此分型标准对于指导临床治疗和判断预后具有现实意义。

Objective：To find practical solutions to the difficulties in typing of acute hepergranular promyelocytic leukemia（M3 ）due to mixed - up of fine and coarse granules. Methods：The proposed typ-ing,based on the percentage of leukemia cells with coarse granules in the cytoplasm,divided M3 into three subtypes：M3a（coarse granule subtype）in which 〉 70% of the leukemia cells contained coarse granules,M3b（ mixed granule subtype）in which 30% to 70% of the leukemia cells contained coarse granules,and M3c（fine granule subtype）in which ≤30% of the leukemia cells contained coarse gran-ules. The relationship between the new typing system and the clinico - pathologic characteristics were studied. Results：Among the 208 cases of APL,M3a ,M3b ,and M3c accounted for 42. 31% ,30. 29%and 27. 4% ,respectively. There was a different sex distribution pattern among the three subtypes,with male dominance in M3a subtype,female dominance in M3c subtype and no sex dominance in M3b subtype. The age distribution pattern was that M3a 〈 M3b 〈 M3c . Fever as the first presenting symptom was most frequently observed in M3b patients,followed by M3a and M3c. Hemorrhage was most frequent in M3a ,fol-lowed by M3b and then M3c. Stern tenderness was observed in some M3a and M3b patients,but not in M3c patients. There are some patients with hepatomegally in some M3a subtypes but not in M3b or M3c sub-types. Splenomegaly was observed in some patients of all the three subtypes（P 〉 0. 05）. Lymph node enlargement was most frequently observed in M3b ,followed by M3a and then M3c . The average levels of peripheral blood hemoglobin,red blood cell count,platelets count and white blood cell count were M3a 〈M3b 〈 M3c. The percentage of peripheral blood promyelocytic leukemia cells was M3c 〉 M3a 〉 M3b. Bone marrow study showed extremely proliferating nuclear cells in M3a subtype,and significantly proliferating nuclear cells in M3b and M3c subtypes. The granulocyte / erythrocyte ratio,promyelocyte percentage, coarse granule promylocyte percentage were M3a 〉 M3b 〉 M3c. Auer bodies were most frequently observed in M3b ,followed by M3c and then M3a. Conclusions：The three subtypes were different in age and sex dis-tribution,clinical signs and symptoms,and peripheral blood and bone marrow smear characteristics. The proposed typing could be of reference value for treatment and prognosis.