Bieniaszewska M, Hellmann A, Kabata J, Rasze-Ja-Specht A
Kliniki Hematologii I.Ch.W., Akademii Medycznej w Gdańsku.
Pol Arch Med Wewn. 1994 Aug;92(2):170-5.
The article presents clinical course analysis of essential thrombocythemia in 17 patients aged 29-82. The diagnostic criteria were the same as described by Polycythemia Vera Study Group. Mean platelet level of diagnosis was 1680 x 10(9)/l. Haemorrhagic complications were observed in 42% of the patients, while thrombotic ones or embolisms in 35%. In two cases both types of complications occurred. Asymptomatic course of the disease was observed in 5 patients. The statistical analysis proved that the patients with platelet count between 900-1900 x 10(9)/l are in danger of developing thrombotic episodes and thus antiaggregation treatment should be considered. If platelet level exceeds 1900 x 10(9)/l the risk of haemorrhage increases, so antiaggregation treatment is contraindicated and thrombocytapheresis is advised instead. The patients was started on treatment when platelet count was above 1000 x 10(9)/l in asymptomatic cases or with lower platelet level in symptomatic ones. The treatment consisted of busulphan, hydroxyurea or interferon alpha (in one of the patients) until lowering platelet level below 600 x 10(9)/l.
本文呈现了17例年龄在29至82岁的原发性血小板增多症患者的临床病程分析。诊断标准与真性红细胞增多症研究组所描述的相同。诊断时的平均血小板水平为1680×10⁹/L。42%的患者出现出血并发症，35%的患者出现血栓形成或栓塞。有2例患者同时出现了这两种并发症。5例患者疾病呈无症状病程。统计分析证明，血小板计数在900 - 1900×10⁹/L之间的患者有发生血栓事件的风险，因此应考虑抗聚集治疗。如果血小板水平超过1900×10⁹/L，出血风险增加，所以抗聚集治疗禁忌，建议采用血小板单采术。无症状患者血小板计数高于1000×10⁹/L或有症状患者血小板水平较低时开始治疗。治疗包括使用白消安、羟基脲或α干扰素（其中1例患者使用），直至血小板水平降至600×10⁹/L以下。
