Weinberg R S, Worsley A, Gilbert H S, Cuttner J, Berk P D, Alter B P
Department of Medicine, Mount Sinai School of Medicine, New York 10029.
Leuk Res. 1989;13(4):331-8. doi: 10.1016/0145-2126(89)90070-2.
The ability of erythroid cultures to distinguish among myeloproliferative disorders was examined. We studied 14 patients with polycythemia vera (PV), 11 with chronic myelogenous leukemia (CML), four with non-PV erythrocytosis, two with agnogenic myeloid metaplasia, as well as three normal fetuses and greater than 25 normal adults. Endogenous, i.e. grew without added erythropoietin, bone marrow CFU-E-derived colonies were observed in all but one PV patient. However, endogenous blood BFU-E-derived bursts were observed in only eight of 14 PV patients. Endogenous erythroid colonies were not seen in cultures from any normal adults or fetuses, or patients with CML, erythrocytosis, or myeloid metaplasia. In PV, relative HbF synthesis was always greater in cultures without erythropoietin, while in cultures from all other patients relative HbF synthesis was similar to that observed in cultures from normal individuals. We conclude that PV and CML are distinguishable in culture since CML patients do not have endogenous growth. Most important, endogenous bone marrow CFU-E-derived colonies are the only consistently unique observation in patients with PV, and endogenous CFU-E- and BFU-E-derived colonies and bursts are not uniformly observed in PV blood cultures. In-vitro studies of erythropoiesis to confirm the diagnosis of PV, therefore, require marrow when endogenous colonies and bursts are absent from blood cultures.
研究了红系培养物区分骨髓增殖性疾病的能力。我们研究了14例真性红细胞增多症(PV)患者、11例慢性粒细胞白血病(CML)患者、4例非PV红细胞增多症患者、2例原因不明的髓样化生患者,以及3例正常胎儿和超过25名正常成年人。除1例PV患者外,在所有患者中均观察到内源性(即不添加促红细胞生成素即可生长)骨髓CFU-E来源的集落。然而,在14例PV患者中,仅8例观察到内源性血液BFU-E来源的爆式集落形成单位。在任何正常成年人、胎儿或CML、红细胞增多症或髓样化生患者的培养物中均未观察到内源性红系集落。在PV中,不添加促红细胞生成素的培养物中相对HbF合成总是更高,而在所有其他患者的培养物中,相对HbF合成与正常个体培养物中观察到的相似。我们得出结论,PV和CML在培养中是可区分的,因为CML患者没有内源性生长。最重要的是,内源性骨髓CFU-E来源的集落是PV患者唯一始终独特的观察结果,并且在PV血液培养物中并非总能观察到内源性CFU-E和BFU-E来源的集落及爆式集落形成单位。因此,当血液培养物中不存在内源性集落和爆式集落形成单位时,对PV诊断进行体外红细胞生成研究需要使用骨髓。