Burkhardt R, Kronseder A
Fortschr Med. 1977 May 19;95(19):1261-6.
Primary thrombocythaemia is to be distinguished from the secondary type by higher counts of megakaryocytes especially of atypic and gigantic forms of these cells, showing up in adequate histological preparations of bone marrow biopsies. From the analysis of those preparations the autonomous proliferation of the megakaryocytopoiesis clearly is to be understood as the reason of the socalled primary thrombocythaemia, occurring in the forms of isolated or mixtcellular megakaryocytic myelosis of the well differentiated type. Both of these forms can convert into thrombocytopenia when atypical and immature megakaryocytes start to overcrowd the picture or when myelofibrosis develops. The latter is to be considered in causal connections with the ineffective dislocated thrombopoiesis, a common finding among megakary-ocytic myelosis complicated with myelofibrosis. Megakaryocytic myelosis therefore is the clue of three different clinical syndromes, which are alternatively marked by the haemostaseologic or the histologic consequences of the overproduction of platelets, or by the proliferation of the megakaryocytes themselves.
原发性血小板增多症与继发性血小板增多症的区别在于巨核细胞数量增多,尤其是这些细胞的非典型和巨大形态,这在骨髓活检的适当组织学切片中可见。通过对这些切片的分析,巨核细胞生成的自主增殖显然被理解为所谓原发性血小板增多症的原因,其以分化良好型的孤立性或混合细胞性巨核细胞骨髓增殖症的形式出现。当非典型和未成熟的巨核细胞开始充斥画面或发生骨髓纤维化时,这两种形式都可转变为血小板减少症。后者应被视为与无效的异位血小板生成有因果关系,这是巨核细胞骨髓增殖症合并骨髓纤维化时的常见发现。因此,巨核细胞骨髓增殖症是三种不同临床综合征的线索,这些综合征交替表现为血小板过度生成的止血或组织学后果,或巨核细胞自身的增殖。