Messinezy M, Sawyer B, Westwood N B, Pearson T C
Division of Haematology, United Medical School, St Thomas' Hospital, London, U.K.
Eur J Haematol. 1994 Sep;53(3):163-7. doi: 10.1111/j.1600-0609.1994.tb00665.x.
25 patients with idiopathic erythrocytosis (absolute increase in red cell mass without conventional criteria of primary polycythaemia or known underlying cause) have been further studied for evidence of primary or secondary polycythaemia. Additional non-conventional criteria used were: platelet distribution width, platelet nucleotide ratio, serum erythropoietin, clinical evidence of ischaemic vascular disease and erythroid culture variables in serum-free system. All had been used in an earlier study in score form to assist in the diagnosis of primary polycythaemia. These patients were also newly assessed for the presence of hypoxia (supine oximeter values, history suggestive of sleep apnoea), for renal lesions and for splenic enlargement (impalpable) by ultrasound or computerized tomography. 7 patients had erythroid culture scores suggesting primary polycythaemia but the addition of non-culture criteria did not result in any scores more strongly predictive of primary polycythaemia. Supine oximeter values < 92% suggested hypoxaemia as the mechanism of polycythaemia in 3 patients in whom it had not previously been suspected. Some splenic enlargement (impalpable) was demonstrated in 6 patients, only 1 of whom had erythroid culture scores suggesting primary polycythaemia. 12 patients had confirmed, raised erythropoietin levels. We conclude that idiopathic erythrocytosis refers to a heterogenous group of patients. Features of primary or secondary polycythaemia may be demonstrated in some of them by additional new study techniques. The raised erythropoietin values found in half the patients were unexpected.
对25例特发性红细胞增多症患者(红细胞容量绝对增加,无原发性红细胞增多症的传统标准或已知潜在病因)进行了进一步研究,以寻找原发性或继发性红细胞增多症的证据。使用的其他非传统标准包括:血小板分布宽度、血小板核苷酸比率、血清促红细胞生成素、缺血性血管疾病的临床证据以及无血清系统中的红系培养变量。所有这些标准在早期研究中都以评分形式使用,以协助原发性红细胞增多症的诊断。这些患者还重新评估了是否存在低氧(仰卧位血氧饱和度值、提示睡眠呼吸暂停的病史)、肾脏病变以及通过超声或计算机断层扫描检查脾脏是否肿大(触诊不到)。7例患者的红系培养评分提示原发性红细胞增多症,但增加非培养标准后,并没有得出更能强烈预测原发性红细胞增多症的评分。仰卧位血氧饱和度值<92%提示3例患者的红细胞增多症机制为低氧血症,此前未怀疑过这一点。6例患者显示有脾脏肿大(触诊不到),其中只有1例患者的红系培养评分提示原发性红细胞增多症。12例患者的促红细胞生成素水平证实升高。我们得出结论,特发性红细胞增多症指的是一组异质性患者。通过额外的新研究技术,在其中一些患者中可能会发现原发性或继发性红细胞增多症的特征。半数患者促红细胞生成素水平升高是出乎意料的。