Lombardo Gérôme, Hafraoui Kaoutar, Beguin Yves, Vertenoeil Gaëlle
Service d'Hématologie clinique, CHU Liège, Belgique.
Rev Med Liege. 2024 Feb;79(2):110-115.
Polycythemia is suspected when hemoglobin and/or hematocrit levels exceed established norms based on gender and age. This biological anomaly can arise from a myeloproliferative neoplasm known as polycythemia vera, or be secondary to excess erythropoietin (EPO) or decreased in plasma volume. Faced with polycythemia, the search for JAK2 mutations and measurement of serum EPO levels can guide toward the etiology. In polycythemia vera, thromboembolic events are the most lethal complications and unfortunately often the initial manifestation of the disease. The condition can also progress to myelofibrosis or acute leukemia. Management aims at reducing the hematocrit below 45 %, in order to limit, but not completely prevent, thrombo-embolic complications. This article elaborates on the clinical considerations around this biological anomaly, relevant complementary examinations, and briefly the therapeutic management.
当血红蛋白和/或血细胞比容水平超过基于性别和年龄确定的标准时，应怀疑患有红细胞增多症。这种生物学异常可能源于一种称为真性红细胞增多症的骨髓增殖性肿瘤，或者继发于促红细胞生成素（EPO）过多或血浆容量减少。面对红细胞增多症，寻找JAK2突变和测量血清EPO水平可有助于明确病因。在真性红细胞增多症中，血栓栓塞事件是最致命的并发症，不幸的是，它往往是该疾病的初始表现。这种情况也可能进展为骨髓纤维化或急性白血病。治疗目标是将血细胞比容降至45%以下，以限制但不能完全预防血栓栓塞并发症。本文阐述了围绕这种生物学异常的临床注意事项、相关的辅助检查，并简要介绍了治疗管理。
