Itoh K, Kashimura T, Kobayashi Y, Yagasaki F, Sakata T, Kawai N, Matsuda A, Kusumoto S, Fukuda M, Ino H, Murohashi I, Jinnai I, Yoshida S, Bessho M, Saitoh M, Hirashima K
First Department of Internal Medicine, Saitama Medical School.
Rinsho Ketsueki. 1999 Feb;40(2):129-34.
A 78-year-old man was diagnosed as leukocytosis in February 1994. Physical examination revealed marked hepatosplenomegaly. A peripheral blood examination disclosed 95,090/microliter leukocytes without hiatus leukemicus, 6.5 g/dl Hb, and 15.0 x 10(4)/microliter platelets. The neutrophil alkaline phosphatase score was 27, and serum VB12 was above 1,600pg/ml. IgG was identified as monoclonal immunoglobulin of type lambda. Bone marrow specimens demonstrated marked granulocytic hyperplasia. Neither the Philadelphia chromosome (Ph1) nor BCR gene rearrangement was detected; hence, the diagnosis of Ph1 (-) chronic myeloid leukemia (CML) was made. The patient was treated with hydroxyurea and low-dose VP-16 with no improvement, and died of pneumonia and sepsis in June 1995. This case was considered to be consistent with atypical CML (aCML) according to the FAB classification because monocytosis was not observed. It seems likely and interesting that the coexistent monoclonal gammopathy and aCML might have arisen from common abnormal hematopoietic stem cells.
一名78岁男性于1994年2月被诊断为白细胞增多症。体格检查发现明显的肝脾肿大。外周血检查显示白细胞计数为95,090/微升，无白血病裂孔，血红蛋白6.5克/分升，血小板15.0×10⁴/微升。中性粒细胞碱性磷酸酶评分为27，血清维生素B12高于1600皮克/毫升。IgG被鉴定为λ型单克隆免疫球蛋白。骨髓标本显示明显的粒细胞增生。未检测到费城染色体（Ph1）和BCR基因重排；因此，诊断为Ph1（-）慢性髓性白血病（CML）。患者接受羟基脲和低剂量VP-16治疗，病情无改善，于1995年6月死于肺炎和败血症。根据FAB分类，该病例被认为符合非典型CML（aCML），因为未观察到单核细胞增多。共存的单克隆丙种球蛋白病和aCML可能源于共同的异常造血干细胞，这似乎很有可能且有趣。
