Mihalj Mario, Titlić Marina, Bonacin Damir, Dogaš Zoran
Department of Neurology, University Hospital Split, Split, Croatia.
Am J Case Rep. 2013 Sep 25;14:385-7. doi: 10.12659/AJCR.884016. eCollection 2013.
Female, 64 FINAL DIAGNOSIS: Polycythemia rubra vera Symptoms: Burning pain • cramps • hypesthesia • itching • paresthesia
Unusual clinical course.
The association between polycythemia vera and peripheral neuropathy has been described previously but only as a late complication and only with sensory axonal polyneuropathy. We presume the cause of polyneuropathy was hypoxia due to higher blood viscosity and dysfunction of platelet aggregation.
We report the cases of 3 female patients with symptoms and signs of slowly progressive sensorimotor axonal polyneuropathy confirmed with clinical and neurographic examination as first complication of polycythemia vera, which progressed to a major complication. Axonal damage was irreversible despite venipuncture.
Polycythemia vera is rarely manifested with symptoms of sensomotor polyneuropathy as the first signs of the disease, and should therefore be recognized by physicians to prevent further axonal damage and major complications of disease by venipuncture or cytostatic therapy.
女性,64岁 最终诊断:真性红细胞增多症 症状:灼痛、痉挛、感觉减退、瘙痒、感觉异常
不寻常的临床病程
真性红细胞增多症与周围神经病变之间的关联此前已有描述,但仅作为晚期并发症,且仅与感觉轴索性多发性神经病变相关。我们推测多发性神经病变的病因是血液黏稠度升高导致的缺氧以及血小板聚集功能障碍。
我们报告了3例女性患者的病例,这些患者经临床和神经电生理检查确诊为患有缓慢进展的感觉运动轴索性多发性神经病变的症状和体征,这是真性红细胞增多症的首发并发症,并进展为主要并发症。尽管进行了静脉穿刺,但轴突损伤仍不可逆。
真性红细胞增多症很少以感觉运动性多发性神经病变的症状作为该病的首发体征,因此医生应予以识别,以便通过静脉穿刺或细胞毒性疗法预防疾病进一步的轴突损伤和主要并发症。