Nissen C, Cornu P, Weber W, Speck B
Schweiz Med Wochenschr. 1978 Oct 14;108(41):1581-3.
Peripheral blood precursor cells from all of 12 patients with polycythaemia vera (PV) formed hemoglobinized colonies in vitro without addition os was strictly epo-dependent in 30 normals and 8 patients with erythrocytosis of other origin. By addition of 1 U epo/ml to the cultures, colony formation was increased up to 5-fold in PV. Untreated patients with a short history had the highest numbers of such epo-responsive precursor cells. In 3 patients with concomitant myelofibrosis, erythroid in vitro growth was abundant, but scattered, single colonies could hardly be identified and their hemoglobinization was poor. This picture did not change with addition of epo, and hemoglobinization did not improve. In vitro grown colonies in PV had morphological abnormalities as compared to colonies grown from normal precursor cells: all stages of erythroid differentiation and up to 50% necrotic cells were found within single colonies. Scattered colonies contained an excess of large, immature, vacuolated erythroblasts. It is concluded that these functionaland morphological abnormalities of in-vitro erythropoiesis are a reliable indicator of PV. Further, transition to myelofibrosis is recognized by a characteristic growth pattern with decreased epo-responsiveness.
12例真性红细胞增多症(PV)患者的外周血前体细胞在体外无需添加促红细胞生成素(EPO)即可形成血红蛋白化集落,而30名正常人和8例其他原因引起的红细胞增多症患者的集落形成则严格依赖EPO。在培养物中添加1 U/ml的EPO后,PV患者的集落形成增加了5倍。病程短且未经治疗的患者中,此类对EPO有反应的前体细胞数量最多。在3例合并骨髓纤维化的患者中,体外红系生长旺盛,但集落分散,难以识别单个集落,且血红蛋白化较差。添加EPO后,这种情况并未改变,血红蛋白化也未改善。与正常前体细胞形成的集落相比,PV患者体外培养的集落在形态上存在异常:单个集落内可见红系分化的各个阶段,以及高达50%的坏死细胞。分散的集落中含有过多的大的、未成熟的、有空泡的成红细胞。结论是,体外红细胞生成的这些功能和形态异常是PV的可靠指标。此外,向骨髓纤维化的转变可通过EPO反应性降低的特征性生长模式来识别。