Gisslinger H, Jeryczynski G, Gisslinger B, Wölfler A, Burgstaller S, Buxhofer-Ausch V, Schalling M, Krauth M-T, Schiefer A-I, Kornauth C, Simonitsch-Klupp I, Beham-Schmid C, Müllauer L, Thiele J
Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria.
Division of Hematology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
Leukemia. 2016 May;30(5):1126-32. doi: 10.1038/leu.2015.360. Epub 2015 Dec 29.
Essential thrombocythemia (ET) is currently diagnosed either by the British Committee of Standards in Haematology (BCSH) criteria that are predominantly based on exclusion and not necessarily on bone marrow (BM) morphology, or the World Health Organization (WHO) criteria that require BM examination as essential criterion. We studied the morphological and clinical features in patients diagnosed according either to the BCSH (n=238) or the WHO guidelines (n=232). The BCSH-defined ET cohort was re-evaluated by applying the WHO classification. At presentation, patients of the BCSH group showed significantly higher values of serum lactate dehydrogenase and had palpable splenomegaly more frequently. Following the WHO criteria, the re-evaluation of the BCSH-diagnosed ET cohort displayed a heterogeneous population with 141 (59.2%) ET, 77 (32.4%) prefibrotic primary myelofibrosis (prePMF), 16 (6.7%) polycythemia vera and 4 (1.7%) primary myelofibrosis. Contrasting WHO-confirmed ET, the BCSH cohort revealed a significant worsening of fibrosis-free survival and prognosis. As demonstrated by the clinical data and different outcomes between WHO-diagnosed ET and prePMF, these adverse features were generated by the inadvertent inclusion of prePMF to the BCSH group. Taken together, the diagnosis of ET without a scrutinized examination of BM biopsy specimens will generate a heterogeneous cohort of patients impairing an appropriate clinical management.
原发性血小板增多症（ET）目前的诊断依据，要么是英国血液学标准委员会（BCSH）的标准，该标准主要基于排除法，不一定基于骨髓（BM）形态学；要么是世界卫生组织（WHO）的标准，该标准要求将骨髓检查作为基本标准。我们研究了根据BCSH标准（n = 238）或WHO指南（n = 232）诊断的患者的形态学和临床特征。通过应用WHO分类法对BCSH定义的ET队列进行了重新评估。初诊时，BCSH组患者的血清乳酸脱氢酶值显著更高，脾肿大可触及的情况更常见。按照WHO标准，对BCSH诊断的ET队列进行重新评估后发现，这是一个异质性群体，其中有141例（59.2%）ET、77例（32.4%）纤维化前原发性骨髓纤维化（prePMF）、16例（6.7%）真性红细胞增多症和4例（1.7%）原发性骨髓纤维化。与WHO确诊的ET相比，BCSH队列显示无纤维化生存期和预后显著恶化。正如WHO诊断的ET和prePMF之间的临床数据和不同结果所表明的那样，这些不良特征是由于无意中将prePMF纳入BCSH组所致。综上所述，在未仔细检查骨髓活检标本的情况下诊断ET，将产生一个异质性的患者群体，不利于进行适当的临床管理。
