Hellmann Andrzej
Department of Hematology and Transplantology, Medical University, Gdańsk, Poland.
Pol Arch Med Wewn. 2008 Dec;118(12):756-60.
Myeloproliferative syndromes (MPS) are clonal proliferation of hematopoietic progenitor cells characterized by proliferation of 1 or a few cell lines such as granulocytic, erythroid, megakaryocytic or mastocytic. These syndromes include: chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, myelofibrosis, chronic eosinophilic leukemia/hypereosinophilic syndrome, chronic neutrophilic leukemia and systemic mastocytosis. Diagnosis of MPS is often difficult due to need of differential diagnosis with reactive proliferation caused by primarily non-hematological factors. Differentiation of individual MPS forms is also difficult because of overlapping of particular clinical or laboratory adnormalities. Discovery of specific molecular aberrations in the last few years facilitates diagnostic procedures. The discovered gene mutations or their fusions are associated with production of proteins possessing tyrosine kinase properties. These discoveries resulted in the successful introduction of the targeted therapy with tyrosine kinase inhibitors in the recent years.
骨髓增殖性综合征（MPS）是造血祖细胞的克隆性增殖，其特征为一种或几种细胞系（如粒细胞系、红系、巨核细胞系或肥大细胞系）的增殖。这些综合征包括：慢性髓性白血病、真性红细胞增多症、原发性血小板增多症、骨髓纤维化、慢性嗜酸性粒细胞白血病/高嗜酸性粒细胞综合征、慢性中性粒细胞白血病和系统性肥大细胞增多症。由于需要与主要由非血液学因素引起的反应性增殖进行鉴别诊断，MPS的诊断通常很困难。由于特定临床或实验室异常的重叠，个体MPS形式的鉴别也很困难。近年来，特定分子异常的发现促进了诊断程序的发展。发现的基因突变或其融合与具有酪氨酸激酶特性的蛋白质的产生有关。这些发现导致近年来成功引入了酪氨酸激酶抑制剂的靶向治疗。
