潜伏型多发性红细胞症的临床和实验室特征。

Q4 Medicine Georgian medical news Pub Date : 2024-07-01
F Khalilova, A Kerimov
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引用次数: 0

摘要

高水平的血红蛋白和血细胞比容是红细胞增多症(PV)最重要的实验室指标之一。然而,在某些情况下,这些指标可能正常或低于正常水平。这种形式的疾病被称为潜伏性或掩盖性多发性红细胞增多症(LPV)。研究发现,血栓出血并发症(THC)在 LPV 患者中比在典型 PV(CPV)患者中更为常见。不同的研究分析了 JAK2 基因突变等位基因负担、血小板增多、白细胞增多水平与 LPV 期间血栓形成发生率之间的关系。其中一些研究结果相互矛盾。这种情况也可能是由于 LPV 患者诊断和治疗的延误造成的。目的:研究潜伏型 PV 的实验室和临床特征:对2019-2020年登记的PV患者进行了分析。其中区分出 LPV 患者。在疾病诊断过程中,进行了一般血液分析、骨髓穿刺活检和组织学检查、外周血和骨髓分子遗传学检查。研究过程中获得的所有数字指标都根据现代建议进行了统计分析。各组指标按变化顺序排列,并计算每个顺序的平均指标、该指标的标准误差、95%置信水平的置信区间(CI-置信区间)。在对各组进行比较时,考虑了完整性标准 p。计算使用ONE-WAY-ANOVA计算软件:我们对 101 名患者进行了研究。结果:我们对 101 名患者进行了研究,其中发现了 36 名潜伏性多血症患者。对 LPV 和 CPV 患者的临床和实验室指标进行了比较。潜伏多血症患者的主诉较少;脾脏较小;血栓并发症较多;血红蛋白、血细胞比容、红细胞计数较低;血小板数量较高;白细胞计数和 JAK2М617F 基因等位基因负荷无统计学差异。与 CPV 患者相比,大部分 LPV 患者属于 THC 高危人群:根据所得结果,可以得出结论:及时、正确地诊断 LPV 非常重要。尽管疾病以潜伏、掩盖的形式传播,但 THC 更容易发生。这可归因于血液中的血小板计数过高以及缺乏对疾病的及时治疗。
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CLINICAL AND LABORATORY CHARACTERISTICS OF THE LATENT FORM OF POLYCYTHEMIA VERA.

A high level of hemoglobin and hematocrit is one of the most important laboratory indicators of polycythemia vera (PV). Nevertheless, in some cases, these indicators may be normal or below the norm. This form of the disease is called latent or masked PV (LPV). It has been found that thrombohemorrhagic complications (THC) are more common in LPV patients than in classic PV (CPV) patients. The relationship between JAK2 gene mutation allele burden, thrombocytosis, leukocytosis level and the occurrence of thrombosis during LPV was analysed in different studies. The results some of them were conflicted. It is also possible that this situation occurs due to the delay in diagnosis and treatment of patients with LPV.

Aim: investigate the laboratory and clinical features of the latent form of PV.

Materials and methods: An analysis of PV patients registered in 2019-2020 was conducted. Out of them patients with LPV were distinguished. During diagnosis of the disease, general blood analysis, trepanobiopsy and histological examination of bone marrow, molecular genetic examination of peripheral blood and bone marrow were performed. All numerical indicators obtained in the course of the research were statistically analyzed taking according to the modern recommendations. Indicators in the groups were arranged in the order of variation, and the average indicator, standard error of this indicator, confidence interval for the 95% confidence level (CI-confidence interval) were calculated for each order. For comparing the groups, the integrity criterion p was taken into account. Calculations were performed using ONE-WAY-ANOVA calculation software.

Results: We study 101 patients. Out of them 36 patients with latent polycythemia were identified. The clinical and laboratory parameters of patients with LPV and CPV were compared. In latent PV the complaints of patients were less intense; the size of the spleen was smaller; thrombotic complications were more often; hemoglobin, hematocrit, erythrocytes count was lower; the number of platelets was higher; leukocytes count and JAK2М617F gene allel burden were not statistically different. The most part of LPV patients, in contrast to CPV patients, was in a high-risk group of THC.

Conclusion: According to the obtained results, it can be concluded that timely and correct diagnosis of LPV is very important. Despite the fact that disease passes in a latent, masked form, THC are more likely to occur. This can be attributed to the high platelet count in the blood and the lack of timely treatment of the disease.

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来源期刊
Georgian medical news
Georgian medical news Medicine-Medicine (all)
CiteScore
0.60
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发文量
207
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