Chronic neutrophilic leukemia associated with monoclonal gammopathy of undetermined significance.
Chronic neutrophilic leukemia associated with monoclonal gammopathy of undetermined significance.
复制标题
慢性中性粒细胞白血病与意义未定的单克隆丙种球蛋白病相关。
DOI：
10.1159/000203863
复制
发表时间：
1996
影响因子：
2.4
通讯作者：
Tsukasa Abe
中科院分区：
文献类型：
--
作者：
Takayoshi Ito;Hiroshi Kojima;Koji Otani;T. Komeno;Shoichi Mitsuhashi;Y. Hasegawa;Toshitaka Kobayashi;Haruhiko Ninomiya;Toshiro Nagasawa;Tsukasa Abe
一位30岁男性慢性中性粒细胞白血病（CNL）合并单克隆伽麻病。入院体格检查显示中度肝脾肿大。初始血细胞计数显示中性粒细胞增多（42.2 x 10(9)/1, 90%为成熟中性粒细胞）。白细胞碱性磷酸酶（LAP）评分升高。骨髓穿刺显示骨髓增生，无发育异常。骨髓细胞核型和分子分析显示费城（Ph1）染色体缺失，bcr基因重排。由于没有潜在的感染或肿瘤，他被诊断为CNL伴IgG卡帕型单克隆γ病（IgG, 1269 mg/dl）。除了与意义不确定的单克隆γ病（MGUS）相关外，本病例在12年随访期间还表现出CNL的自发缓解，并伴有血清IgG水平逐渐升高至3,000 mg/dl。据我们所知，文献中有19例CNL与单克隆γ病相关。这些病例的中位生存期为5年。虽然只有6例CNL合并MGUS，但这些病例的生存率特别好。结合观察到本病例的白细胞增多和肝脾肿大在没有特异性治疗的情况下消退，我们推测本病例的骨髓增生可能是对潜在的单克隆γ病变的类白血病反应。
A 30-year-old man with chronic neutrophilic leukemia (CNL) in association with monoclonal gammopathy is presented. Physical examination on admission revealed moderate hepatosplenomegaly. Initial blood count showed neutrophilic leukocytosis (42.2 x 10(9)/1 with 90% mature neutrophils). Leukocyte alkaline phosphatase (LAP) score was elevated. Bone marrow aspiration showed myeloid hyperplasia without dysplastic features. Karyotypic and molecular analyses of bone marrow cells showed the absence of Philadelphia (Ph1) chromosome and bcr gene rearrangement. Because there was no underlying infection or neoplasm, he was diagnosed as having CNL associated with IgG kappa-type monoclonal gammopathy (IgG, 1,269 mg/dl). In addition to its association with monoclonal gammopathy of undetermined significance (MGUS), the present case was also characterized by spontaneous remission of CNL during the 12-year follow-up, accompanied by a gradual increase in serum IgG levels up to 3,000 mg/dl. As far as we know, there have been 19 cases of CNL associated with monoclonal gammopathy in the literature. The median survival of these cases was 5 years. Although there have been only 6 cases of CNL associated with MGUS, survival of these cases was particularly favorable. Taken together with the observation that leukocytosis and hepatosplenomegaly in the present case subsided without specific treatment, we speculate that myeloid proliferation in the present case may have been a leukemoid reaction to underlying monoclonal gammopathy.