Rigolin G M, Cuneo A, Roberti M G, Bardi A, Castoldi G
Dipartimento di Scienze Biomediche e Terapie Avanzate, Università di Ferrara, Italy.
Haematologica. 1997 Jan-Feb;82(1):25-30.
Patients with myelodysplastic syndromes (MDS) showing high numbers of abnormally localized immature precursors (ALIP) also display myelomonocytic antigens on immature cells, and it has been suggested that the presence of monocytosis could define a distinct subset of MDS characterized by poorer survival. The objective of this study was to analysis the incidence and significance of monocytosis among our series of patients with MDS and correlate the distributions of these elements with other hematologic features.
We evaluated the monocytic component in myelodysplastic syndromes in order to clarify the significance of monocytosis in MDS and its relationship with CMMoL and other MDS. Monocytosis was defined as a percentage of blood monocytes greater than 10%.
Among a series of 139 consecutive MDS patients, we describe a group of 29 (20.8%) patients with monocytosis and dysplastic features involving multiple cell lineages which do not fulfill the criteria for diagnosis of CMMoL or aCML. These patients, who do not differ from MDS without monocytosis in the main clinical parameters, are characterized by relatively higher leukocyte (WBC 6.6 x 10(9)/L) and granulocyte counts (PMN 2.5 x 10(9)/L), hypercellular bone marrow and relatively poor prognosis. Among these patients, we observed a particularly high incidence of evolution to CMMoL (34.5%) and AML (17.2%) with monocytic component (FAB M4 and M5). Cytogenetic data demonstrated clonal chromosome changes in 11/13 patients with MDS and monocytosis, while only 19/41 patients without monocytosis showed clonal abnormalities.
The combination of hematologic and cytogenetic features in our study suggests that it is reasonable to consider myelodysplasia with monocytosis as a distinct disease subset of MDS, characterized by multilineage dysplasia along with a higher incidence of karyotype aberrations. The multi-step pathogenetic process in these patients may have reached a more advanced stage at which the relative or absolute increase in the number of monocytes may represent the first event in the subsequent progression of the disease towards acute leukemia.
骨髓增生异常综合征（MDS）患者若显示大量异常定位的幼稚前体细胞（ALIP），其幼稚细胞上也会表达髓单核细胞抗原，并且有人提出单核细胞增多症的存在可能定义了一个具有较差生存率特征的MDS独特亚组。本研究的目的是分析我们系列MDS患者中单核细胞增多症的发生率及意义，并将这些指标的分布与其他血液学特征相关联。
我们评估了骨髓增生异常综合征中的单核细胞成分，以阐明单核细胞增多症在MDS中的意义及其与慢性粒单核细胞白血病（CMMoL）和其他MDS的关系。单核细胞增多症定义为血液中单核细胞百分比大于10%。
在连续的139例MDS患者中，我们描述了一组29例（20.8%）有单核细胞增多症且发育异常特征累及多个细胞系的患者，这些患者不符合CMMoL或非典型慢性粒细胞白血病（aCML）的诊断标准。这些患者在主要临床参数上与无单核细胞增多症的MDS患者无差异，其特征为白细胞（WBC 6.6×10⁹/L）和粒细胞计数相对较高（PMN 2.5×10⁹/L）、骨髓细胞增多且预后相对较差。在这些患者中，我们观察到向伴有单核细胞成分（FAB M4和M5）的CMMoL（34.5%）和急性髓系白血病（AML，17.2%）演变的发生率特别高。细胞遗传学数据显示，13例有单核细胞增多症的MDS患者中有11例存在克隆性染色体改变，而无单核细胞增多症的41例患者中只有19例显示克隆性异常。
我们研究中的血液学和细胞遗传学特征组合表明，将伴有单核细胞增多症的骨髓增生异常视为MDS的一个独特疾病亚组是合理的，其特征为多系发育异常以及较高的核型畸变发生率。这些患者的多步骤致病过程可能已达到一个更 advanced 阶段，此时单核细胞数量的相对或绝对增加可能代表疾病随后向急性白血病进展的首个事件。 （注：“advanced”此处结合语境似应为“晚期、进展期”之类意思，但按要求未做调整）
