Koopmans S M, van Marion A M W, Schouten H C
Department of Pathology of the University Hospital Maastricht, Maastricht, the Netherlands.
Neth J Med. 2012 May;70(4):159-67.
Essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome-negative myeloproliferative neoplasia (Ph- MPN). MPNs are clonal bone marrow stem cell disorders characterised by a proliferation of one or more of the myeloid, erythroid or megakaryocytic cell lines. Due to the different affected cell lines, MPNs show typical clinical and histological features. In 2005, a mutation in the JAK2 gene was discovered which generated more insight into the pathogenetic working mechanism of MPNs. However, the treatment of MPN patients is still mainly only palliative, although progress in reducing the symptoms of MPN patients has been made. This review will give a general overview of MPN patients for internal medicine physicians.
原发性血小板增多症(ET)、真性红细胞增多症(PV)和原发性骨髓纤维化(PMF)属于费城染色体阴性骨髓增殖性肿瘤(Ph- MPN)。骨髓增殖性肿瘤是克隆性骨髓干细胞疾病,其特征是一种或多种髓系、红系或巨核细胞系增殖。由于受影响的细胞系不同,骨髓增殖性肿瘤表现出典型的临床和组织学特征。2005年,发现了JAK2基因突变,这使人们对骨髓增殖性肿瘤的发病机制有了更多了解。然而,尽管在减轻骨髓增殖性肿瘤患者症状方面取得了进展,但对骨髓增殖性肿瘤患者的治疗仍然主要只是姑息性的。本综述将为内科医生提供骨髓增殖性肿瘤患者的总体概述。