Vodoff M V, Nelken B, Vic P, Farriaux J P
Service de pédiatrie, gastroentérologie et génétique médicale, CHRU de Lille, France.
Arch Pediatr. 1996 Sep;3(9):870-3. doi: 10.1016/0929-693x(96)87575-0.
Polycythemia vera is a very rare disease in childhood; its treatment for this reason is not well established.
An 11-year-old boy was admitted for polycythemia vera. The three classical major criteria for this condition were present and no cause for secondary polyglobulia could be recognized. The patient was given hydroxyurea. Three years later, he is well with hematocrit below 50%.
Oral continuous myelosuppressive therapy with hydroxyurea may be proposed in children if there is no HLA-matched family member. Long-term follow-up is nevertheless necessary in order to detect relapse or side-effects of the treatment.
真性红细胞增多症在儿童中是一种非常罕见的疾病；因此其治疗方法尚未完全确立。
一名11岁男孩因真性红细胞增多症入院。该病症的三项经典主要标准均存在，且未发现继发性红细胞增多的病因。患者接受了羟基脲治疗。三年后，他情况良好，血细胞比容低于50%。
如果没有 HLA 匹配的家庭成员，对于儿童真性红细胞增多症可考虑采用羟基脲进行口服持续骨髓抑制治疗。然而，仍需要进行长期随访以检测治疗的复发情况或副作用。
