Gianelli Umberto, Vener Claudia, Raviele Paola Rafaniello, Moro Alessia, Savi Federica, Annaloro Claudio, Somalvico Francesco, Radaelli Franca, Franco Vito, Deliliers Giorgio Lambertenghi
II Cattedra di Anatomia Patologica, Dipartimento di Medicina, Chirurgia e Odontoiatria, Università degli Studi di Milano, A.O. San Paolo, Via Di Rudini 8, 20142 Milan, Italy,
Leuk Lymphoma. 2006 Sep;47(9):1774-81. doi: 10.1080/10428190600678975.
We reviewed a large series of patients with essential thrombocythemia diagnosed on the basis of the Polycythemia Vera Study Group criteria, and reclassified them by evaluating their major morphologic features and clinical course using the World Health Organization classification. The morphologic review of the bone marrow biopsies of 116 patients (44 males and 72 females; aged 19 - 83 years, median 55 years; median follow-up 121 months) led to 22 cases (19%) being classified as essential thrombocythemia (ET), 24 (21%) as chronic idiopathic myelofibrosis (CIMF)-0, 44 (37%) as CIMF-1, 13 (12%) as CIMF-2, 9 (8%) as latent phase polycythemia vera, and four (3%) as chronic myeloproliferative disorder, unclassifiable. There was a significant difference in the median age of the ET and fibrotic CIMF patients (54.7 +/- 13.55 vs. 59.13 +/- 15.05 years; P = 0.03). Histologic analysis showed that the simultaneous presence of loose clusters of large/giant megakaryocytes and nuclear hyperlobulation was significantly different between the ET and the prefibrotic CIMF (P<0.01) and fibrotic CIMF patients (P<0.01), and that the association of dense clusters of megakaryocytes with maturation defects and bulbous nuclei also distinguished the prefibrotic CIMF (P<0.05) and fibrotic CIMF patients (P<0.001) from those with ET. The association of cellularity, granulocytic proliferation and reticulin fibers was helpful in distinguishing prefibrotic from fibrotic CIMF (P<0.001).
我们回顾了一大组根据真性红细胞增多症研究组标准诊断为原发性血小板增多症的患者，并根据世界卫生组织分类对其主要形态学特征和临床病程进行评估后重新分类。对116例患者（44例男性和72例女性；年龄19 - 83岁，中位年龄55岁；中位随访121个月）的骨髓活检进行形态学评估，结果显示22例（19%）被分类为原发性血小板增多症（ET），24例（21%）为慢性特发性骨髓纤维化（CIMF）-0，44例（37%）为CIMF-1，13例（12%）为CIMF-2，9例（8%）为隐匿期真性红细胞增多症，4例（3%）为无法分类的慢性骨髓增殖性疾病。ET患者和纤维化CIMF患者的中位年龄存在显著差异（54.7±13.55岁 vs. 59.13±15.05岁；P = 0.03）。组织学分析表明，ET患者与纤维化前期CIMF患者（P<0.01）和纤维化CIMF患者（P<0.01）相比，大/巨型巨核细胞松散簇和核分叶过多同时出现的情况有显著差异，并且巨核细胞致密簇与成熟缺陷和球状核的关联也使纤维化前期CIMF患者（P<0.05）和纤维化CIMF患者（P<0.001）与ET患者区分开来。细胞密度、粒细胞增殖和网状纤维的关联有助于区分纤维化前期和纤维化CIMF（P<0.001）。
