Thiele Juergen, Kvasnicka Hans Michael
Institute of Pathology, University of Cologne, Cologne, Germany.
Semin Thromb Hemost. 2006 Apr;32(3):219-30. doi: 10.1055/s-2006-939433.
Thrombocythemias with the presenting or developing complications of thromboembolic and hemorrhagic episodes may be encountered at strikingly different incidences in each subtype of chronic myeloproliferative disorders. A critical reappraisal of the Polycythemia Vera Study Group (PVSG) criteria for essential thrombocythemia (ET) reveals that differentiation is explicitly focused on the exclusion of chronic myeloid leukemia and polycythemia vera (PV), but not on prodromal stages of chronic idiopathic myelofibrosis (CIMF) or latent (initial) PV. Consequently, it may be assumed that most series of patients with so-called ET include a considerable fraction of patients with the latter entities. The diagnostic impact of bone marrow (BM) histopathology was recognized by the World Health Organization classification, which emphasizes for the first time positive criteria for ET. The need of a more accurate ET diagnosis is obvious, in particular regarding therapeutic strategies and outcome (i.e., progression into myelofibrosis and blastic crisis). Conversely, early CIMF with accompanying thrombocythemia mimicking (true) ET is characterized by a higher rate of evolution into myelofibrosis and fatal complications. A scrutinized discrimination of thrombocythemias resulting in a clear-cut diagnosis of true versus false ET is warranted by a professional evaluation of BM biopsies in ongoing and prospective clinical trials.
在慢性骨髓增殖性疾病的每种亚型中，出现或发展为血栓栓塞和出血发作并发症的血小板增多症的发生率可能有显著差异。对真性红细胞增多症研究组（PVSG）关于原发性血小板增多症（ET）的标准进行严格重新评估后发现，其鉴别明确侧重于排除慢性髓系白血病和真性红细胞增多症（PV），而非慢性特发性骨髓纤维化（CIMF）的前驱阶段或潜伏性（初始）PV。因此，可以推测，大多数所谓ET患者系列中包括相当一部分患有后两种疾病的患者。骨髓（BM）组织病理学的诊断影响已得到世界卫生组织分类的认可，该分类首次强调了ET的阳性标准。显然需要更准确的ET诊断，特别是在治疗策略和结果（即进展为骨髓纤维化和原始细胞危象）方面。相反，伴有血小板增多症的早期CIMF模仿（真性）ET，其特征是发展为骨髓纤维化和致命并发症的发生率更高。在正在进行的前瞻性临床试验中，通过对BM活检进行专业评估，对血小板增多症进行仔细鉴别以明确诊断真性与假性ET是必要的。
