Danish E H, Rasch C A, Harris J W
Am J Hematol. 1980;9(4):421-8. doi: 10.1002/ajh.2830090409.
A patient presented at 5 years of age with polycythemia vera. He subsequently developed splenic infarctions and died at 20 years of age following cerebral hemorrhage and infarctions. Two months before his death, he developed hypertension and had biochemical evidence of primary hyperparathyroidism and possibly pheochromocytoma. Only nine reported childhood cases of polycythemia vera fulfill the criteria of the Polycythemia Vera Study Group. These cases are summarized and the complications discussed. Although none have progressed to myeloid metaplasia or acute leukemia, these patients are at risk of developing thrombo-hemorrhagic complications; available evidence indicates that they should be managed to keep the hematocrit between 40 and 45%.
一名5岁的真性红细胞增多症患者前来就诊。随后他出现了脾梗死，并在20岁时因脑出血和梗死死亡。在他去世前两个月，他患上了高血压，并有原发性甲状旁腺功能亢进以及可能的嗜铬细胞瘤的生化证据。仅有9例已报道的儿童真性红细胞增多症病例符合真性红细胞增多症研究组的标准。对这些病例进行了总结并讨论了并发症。尽管尚无病例进展为骨髓化生或急性白血病，但这些患者有发生血栓-出血并发症的风险；现有证据表明，应对他们进行治疗以使血细胞比容保持在40%至45%之间。
