Bellucci S, Janvier M, Tobelem G, Flandrin G, Charpak Y, Berger R, Boiron M
Cancer. 1986 Dec 1;58(11):2440-7. doi: 10.1002/1097-0142(19861201)58:11<2440::aid-cncr2820581115>3.0.co;2-y.
This retrospective study reviewed 94 patients, aged 6 to 90 years, with thrombocythemia. The women/men ratio was 1.76. At onset or, less commonly, during the course of the disease, 45% of the patients presented hemorrhagic manifestations, 29% thrombotic complications, and only 14% the association of hemorrhage and thrombosis. The average platelet count was 1200 X 10(9)/1, with no difference according to sex or age. Platelet hypoaggregation was seen in practically all the cases (94%), although myelofibrosis was less frequent (54%). The frequency of hemorrhages increased when the platelet count was above 1000 X 10(9)/1 (P less than 0.01), but the occurrence of thrombosis was correlated neither with platelet count nor with thrombopathy. Survival time was lengthy: 80% of survival (standard error 6%) was observed at 100 months. Transformation to acute leukemia was observed in five patients. Because the disease course is most often prolonged, therapeutic measures must be conservative: anti-aggregating drugs in small doses, and chemotherapy beginning with nonalkylating agents.
这项回顾性研究对94例年龄在6至90岁之间的血小板增多症患者进行了评估。女性与男性的比例为1.76。在疾病发作时，或较少见地在病程中，45%的患者出现出血表现，29%出现血栓并发症，仅有14%同时出现出血和血栓。平均血小板计数为1200×10⁹/L，在性别和年龄方面无差异。几乎所有病例（94%）均出现血小板聚集功能低下，不过骨髓纤维化的发生率较低（54%）。当血小板计数高于1000×10⁹/L时，出血频率增加（P＜0.01），但血栓形成的发生与血小板计数及血小板病均无关联。生存时间较长：在100个月时观察到80%的生存率（标准误差6%）。有5例患者发生了向急性白血病的转化。由于病程通常较长，治疗措施必须保守：小剂量抗聚集药物，以及从非烷化剂开始的化疗。
