Kurata H, Hirai M, Miwa A, Murai Y, Mori M
Department of Hematology, Jichi Medical School.
Rinsho Ketsueki. 1990 Dec;31(12):1906-13.
We examined fourteen patients with chronic myelomonocytic leukemia (CMMoL) according to the following staging criteria at diagnosis; Group A: bone marrow (BM) blast less than 5% (eight cases), Group B; BM blast more than 5% and less than 30% (five cases), Group C; BM blast more than 30% (one case). Compared with Group A, Group B patients have much more peripheral blood leukocyte, granulocyte and monocyte counts, LDH level, and serum and urine lysozyme levels. Two of the five Group B cases transformed to acute leukemia (BC) within one and a half year, and other three patients died of infection and hemorrhage within a year. On the contrary, three of the eight Group A patients survived four years, and transformation to acute leukemia occurred in only one case after four years. Autopsy revealed multiple organ infiltration of monocytoid granulocytes on the patients with advanced stage and more bone marrow blasts. Two cases have coexistence of myeloproliferative disorders, one with essential thrombocythemia, and another with myelofibrosis, which, later, transformed to acute leukemia. And a Group C patient transformed to chronic phase with chemotherapy, and maintained the state for six years, but at the end stage, mature monocytes increased and pancytopenia developed. These findings indicate the heterogeneity of CMMoL in respect of the disease stage and the coexistence of other myeloproliferative disorders.
我们根据以下诊断分期标准对14例慢性粒单核细胞白血病（CMMoL）患者进行了检查；A组：骨髓（BM）原始细胞少于5%（8例），B组：BM原始细胞多于5%且少于30%（5例），C组：BM原始细胞多于30%（1例）。与A组相比，B组患者的外周血白细胞、粒细胞和单核细胞计数、乳酸脱氢酶（LDH）水平以及血清和尿液溶菌酶水平更高。B组的5例患者中有2例在一年半内转化为急性白血病（BC），其他3例患者在一年内死于感染和出血。相反，A组的8例患者中有3例存活了4年，4年后仅1例发生急性白血病转化。尸检显示晚期患者和骨髓原始细胞较多的患者存在单核样粒细胞的多器官浸润。2例患者合并骨髓增殖性疾病，1例合并原发性血小板增多症，另1例合并骨髓纤维化，后者后来转化为急性白血病。1例C组患者经化疗转化为慢性期，并维持该状态6年，但在末期，成熟单核细胞增多且全血细胞减少。这些发现表明CMMoL在疾病分期和其他骨髓增殖性疾病共存方面存在异质性。
