Cripe L D, Hromas R
Walther Oncology Center, Indiana University Medical Center, Indianapolis 46202, USA.
Semin Hematol. 1998 Jul;35(3):200-9.
Among hematologic malignancies, there are three disorders whose behavior is governed principally by the abnormal proliferation of a malignant megakaryocytic clone or a product thereof. These disorders are essential thrombocythemia, agnogenic myeloid metaplasia, and acute megakaryoblastic leukemia. Although there are other myeloproliferative diseases that can have high platelet counts, the major pathobiology of those diseases most commonly results from the proliferation of other hematopoietic lineages. Despite the rarity of the three disorders of malignant megakaryopoiesis mentioned above, advances have been made in the diagnosis, prognosis, and treatment of these disorders in recent years. However, understanding of the cellular and especially molecular pathobiology lags far behind.
在血液系统恶性肿瘤中，有三种疾病的发生主要由恶性巨核细胞克隆或其产物的异常增殖所主导。这些疾病是原发性血小板增多症、特发性骨髓化生和急性巨核细胞白血病。虽然其他骨髓增殖性疾病也可能有血小板计数升高的情况，但这些疾病的主要病理生物学特征通常是由其他造血谱系的增殖引起的。尽管上述三种恶性巨核细胞生成疾病较为罕见，但近年来在这些疾病的诊断、预后和治疗方面已经取得了进展。然而，对细胞尤其是分子病理生物学的理解仍远远落后。
