Department of Haematology, University of Cambridge, 1Cambridge Institute for Medical Research, Cambridge, United Kingdom.
Haematologica. 2010 Nov;95(11):1960-3. doi: 10.3324/haematol.2010.026708. Epub 2010 Jun 9.
Primary myelofibrosis shows histological and pathogenetic overlap with essential thrombocythemia and polycythemia vera. Several diagnostic classifications have been proposed for primary myelofibrosis, although little is known about their clinical utility. In a comparison of three recent classifications, overall concordance was 79%. Inclusion of raised serum lactate dehydrogenase categorized 9% of patients as primary myelofibrosis when other criteria were not met. Although mean serum lactate dehydrogenase levels were higher in patients with primary myelofibrosis, levels were also increased in the majority of patients with essential thrombocythemia or polycythemia vera, and significant overlap was observed. A positive correlation with higher leukocyte and platelet count, and disease duration in primary myelofibrosis, suggests that serum lactate dehydrogenase is a biomarker for disease bulk and/or cellular proliferation. In conclusion, raised lactate dehydrogenase lacks specificity for primary myelofibrosis, consistent with the concept of a phenotypic continuum between essential thrombocythemia, polycythemia vera and primary myelofibrosis.
原发性骨髓纤维化与原发性血小板增多症和真性红细胞增多症在组织学和发病机制上存在重叠。已经提出了几种原发性骨髓纤维化的诊断分类,但对其临床实用性知之甚少。在最近的三种分类的比较中,总一致性为 79%。当其他标准不满足时,升高的血清乳酸脱氢酶将 9%的患者归类为原发性骨髓纤维化。虽然原发性骨髓纤维化患者的平均血清乳酸脱氢酶水平较高,但在大多数原发性血小板增多症或真性红细胞增多症患者中也升高,并且观察到显著的重叠。与原发性骨髓纤维化中较高的白细胞和血小板计数以及疾病持续时间呈正相关,表明血清乳酸脱氢酶是疾病负荷和/或细胞增殖的生物标志物。总之,升高的乳酸脱氢酶对原发性骨髓纤维化缺乏特异性,这与原发性血小板增多症、真性红细胞增多症和原发性骨髓纤维化之间存在表型连续统的概念一致。