Geary C G, Catovsky D, Wiltshaw E, Milner G R, Scholes M C, Van Noorden S, Wadsworth L D, Muldal S, MacIver J E, Galton D A
Br J Haematol. 1975 Jul;30(3):289-302. doi: 10.1111/j.1365-2141.1975.tb00544.x.
The natural history and haematological features of 18 patients with a chronic form of myelomonocytic leukaemia are described. The majority were elderly and, in this series, females predominated. Haematological prodomata, such as unexplained monocytosis, leucopenia, or thrombocytopenia were common, and the clinical onset was insidious. Splenomegaly was variable but tended to increase as the disease progressed. Anaemia was usually less than in the acute disease, unless compounded by iron deficiency. The blood film typically showed a mixed monocytosis and granulocytosis, cells in both lines showing abnormalities. 'Paramyeloid' cells, appearing in Romanowsky stained films intermediate between myelocytes and monocytes, were characteristic, although cytochemical and electron microscopical analysis suggests that these cells may be allotted to one or other cell line. The marrow aspirate was characteristically hypercellular, showed granulocytic hyperplasia, and, in contrast to the well-differentiated blood picture, the proportion of poorly differentiated cells, including blasts, was high. Serum lysozyme levels were usually raised. Five of the 18 cases survived more than 5 years, while 10 lived 2 years or longer. The morphological and clinical features form part of a spectrum including acute myelomonocytic leukaemia, into which several of the patients transformed. Recognition of the syndrome is important because the patients are probably best managed without intensive chemotherapy.
本文描述了18例慢性粒单核细胞白血病患者的自然病史和血液学特征。大多数患者为老年人,在本系列中女性占主导。血液学前驱症状,如不明原因的单核细胞增多、白细胞减少或血小板减少很常见,且临床起病隐匿。脾肿大程度不一,但往往随着疾病进展而增大。除非合并缺铁,贫血通常不如急性疾病严重。血涂片通常显示单核细胞增多和粒细胞增多混合存在,两系细胞均显示异常。“副髓细胞”出现在罗曼诺夫斯基染色片中,介于髓细胞和单核细胞之间,具有特征性,尽管细胞化学和电子显微镜分析表明这些细胞可能归属于某一细胞系。骨髓穿刺物的特点是细胞增多,显示粒细胞增生,与分化良好的血象相反,包括原始细胞在内的分化不良细胞比例很高。血清溶菌酶水平通常升高。18例患者中有5例存活超过5年,10例存活2年或更长时间。形态学和临床特征构成了一个谱系的一部分,包括急性粒单核细胞白血病,其中有几名患者转变为此病。认识该综合征很重要,因为这些患者可能最好不进行强化化疗。