Campr V
Ustav potologie a molekulární mediciny 2. LF UK a FN Motol, Praha.
Cesk Patol. 2011 Jul;47(3):84-93.
A histological picture in pretreatment bone marrow trephine biopsy is an essential part of Ph-negative myeloproliferative neoplasm diagnosis according to WHO classification. Polycythaemia vera is histologically defined as a hypercellular trilinear myeloproliferation. Hypercellular haematopoiesis with granulocytic and megakaryocytic proliferation is typical for primary myelofibrosis. In essential thrombocythaemia the haematopoiesis is normocellular with proliferation of megakaryocytes only. The most important differential diagnostic features are morphology and distribution of megakaryocytes, and presence of fibrosis. In primary myelofibrosis there are typically ,dysplastic" megakaryocytes forming tight (dense) clusters, and variable extent of fibrosis, while mature megakaryocytes forming loose clusters and no fibrosis are found in essential thrombocythaemia. In reactive thrombocytosis and erythrocytosis the number of normally appearing megakaryocytes is not increased and they are not forming clusters. Prodromal (latent) phases of myeloproliferative neoplasms often unrecognized by recent WHO classification criteria are discussed as well as a differential diagnosis of myeloproliferative disorders associated with thrombocytosis.
根据世界卫生组织（WHO）分类，治疗前骨髓穿刺活检的组织学图像是Ph阴性骨髓增殖性肿瘤诊断的重要组成部分。真性红细胞增多症在组织学上被定义为一种细胞增多的三系骨髓增殖。原发性骨髓纤维化的典型表现是细胞增多的造血，伴有粒细胞和巨核细胞增殖。原发性血小板增多症的造血是正常细胞性的，仅伴有巨核细胞增殖。最重要的鉴别诊断特征是巨核细胞的形态和分布以及纤维化的存在。在原发性骨髓纤维化中，通常存在“发育异常”的巨核细胞形成紧密（密集）的簇，以及不同程度的纤维化，而在原发性血小板增多症中则发现成熟的巨核细胞形成松散的簇且无纤维化。本文还讨论了骨髓增殖性肿瘤的前驱（潜伏）期（这一阶段常未被WHO最新分类标准所认识）以及与血小板增多症相关的骨髓增殖性疾病的鉴别诊断。
