Ellis J T, Peterson P
Pathol Annu. 1979;14 Pt 1:383-403.
The sequential biopsies and the careful clinical and laboratory studies in this large prospective study of PV patients offer a unique opportunity to properly evaluate the diagnostic and prognostic importance of the biopsy and to study the complications of this condition. The results of the study to date confirm and extend previous studies. Because of the long natural history of PV, the results of studies relating to leukemia, other myeloproliferative diseases, myelofibrosis, and effects of therapy are tentative at this time, even though the study is in its eleventh year. Hypercellularity of the marrow, together with hyperplasia and hypertrophy of megakaryocytes, is an almost constant finding in untreated PV. A very few cases (7 of 281) had relatively normal cellularities and normal megakaryocytic concentrations. Whether these findings were the result of sampling errors could not be determined, since only one site was biopsied. In any event, we found no unique clinical or laboratory features to distinguish these patients. At this time, the course of these patients appears to be the same as that of the other patients. Although increases in reticulin were regularly found during the spent phase of polycythemia, the relationship was not a precise one. For example, a moderate to marked increase in reticulin was found in 12 percent of the patients early in the course of the disease and was not predictive that the spent phase with myeloid metaplasia was imminent. In addition, in a given patient with serial biopsies taken over several years, some variability in reticulin was noted among the biopsies. Whether this represented variation in sampling or fluctuation in reticulin content could not be decided at this time. Using the standard criteria for examination of the marrows, we have found it impossible to predict which patients will develop leukemia, since the pretreatment and posttreatment biopsies almost up to the clinical onset cannot be separated from the remainder of the group.
在这项针对真性红细胞增多症（PV）患者的大型前瞻性研究中，序贯活检以及细致的临床和实验室研究提供了一个独特的机会，能够恰当地评估活检在诊断和预后方面的重要性，并研究该病症的并发症。迄今为止的研究结果证实并拓展了先前的研究。鉴于PV的自然病程较长，即便该研究已进入第十一年，但目前有关白血病、其他骨髓增殖性疾病、骨髓纤维化以及治疗效果的研究结果仍具有不确定性。骨髓细胞增多，以及巨核细胞的增生和肥大，在未经治疗的PV中几乎是恒定的表现。极少数病例（281例中有7例）的细胞计数相对正常，巨核细胞浓度也正常。由于仅对一个部位进行了活检，所以无法确定这些结果是否是抽样误差所致。无论如何，我们未发现可区分这些患者的独特临床或实验室特征。目前，这些患者的病程似乎与其他患者相同。尽管在红细胞增多症的消耗期经常发现网硬蛋白增加，但这种关系并不精确。例如，在疾病早期，12%的患者出现了中度至显著的网硬蛋白增加，但这并不能预测即将进入伴有髓外化生的消耗期。此外，在对同一患者进行数年的系列活检中，各次活检之间的网硬蛋白存在一定差异。目前尚无法确定这是抽样差异还是网硬蛋白含量的波动。使用骨髓检查的标准标准，我们发现无法预测哪些患者会发展为白血病，因为直至临床发病前的治疗前和治疗后活检结果与该组其他患者的结果几乎无法区分。
