Department of Leukemia, the University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Section of Benign Hematology, the University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Expert Rev Hematol. 2023 Apr;16(4):245-251. doi: 10.1080/17474086.2023.2192475. Epub 2023 Mar 23.
Erythrocytosis is associated with an elevation of the hemoglobin level above 16.5 g/dL in men and above 16 g/dL in women and an elevation of the hematocrit level above 49% in men and > 48% in women. In primary erythrocytosis, the defect is a clonal disorder in the myeloid compartment of the bone marrow, leading to an increased red cell production. Secondary erythrocytosis is the result of external stimuli to the bone marrow, leading to the production of red cells in excess. Secondary erythrocytosis is more common than primary erythrocytosis and has a broad differential diagnosis.
This review will discuss secondary erythrocytosis, its causes, clinical presentation, and both diagnostic and therapeutic approaches.
Although secondary erythrocytosis is more common than PV, there are still challenges and difficulties associated with the distinction between these two conditions. Moreover, there is a paucity of data and guidance when it comes to the management of certain congenital and acquired conditions. A pragmatic approach is recommended in order to identify the cause for this condition. Treatment should be directed at the management of the underlying cause.
红细胞增多症是指男性血红蛋白水平高于 16.5 g/dL，女性高于 16 g/dL，男性血细胞比容水平高于 49%，女性高于 48%。原发性红细胞增多症是骨髓髓系的克隆性疾病，导致红细胞生成增加。继发性红细胞增多症是骨髓受到外部刺激的结果，导致红细胞生成过多。继发性红细胞增多症比原发性红细胞增多症更为常见，且具有广泛的鉴别诊断。
本文将讨论继发性红细胞增多症的病因、临床表现、诊断和治疗方法。
虽然继发性红细胞增多症比 PV 更为常见，但在这两种疾病的鉴别诊断方面仍存在挑战和困难。此外，在某些先天性和获得性疾病的治疗方面，数据和指导仍然匮乏。建议采取实用的方法来确定该病症的病因。治疗应针对潜在病因进行。
