Simon M, Jouet J P, Huart J J, Demory J L, Bauters F
Sem Hop. 1984 Apr 19;60(17):1173-9.
The 61 observations of primary thrombocythemia described in this report represent approximately 15% of the cases of polycythemia vera recorded by the authors over the same 18-year period. The group includes 35 females and 26 males, with a mean age of 62. The disease is usually discovered on routine blood tests (half of cases), and more rarely because of hemorrhagic or thrombotic manifestations. Splenomegaly is found in one-third of cases. Platelet count is permanently above 800 X 10(9)/l (mean : 1 500 X 10(9)/l); mild hyperleukocytosis (mean : 16 X 10(9)/l) with predominant neutrophil polynuclears is usual but myelemia is not constant (28% of cases) and always very moderate; red cell parameters are normal in three-fourths of cases, while the remaining patients have anemia, either due to iron depletion or not. Reticulinic myelofibrosis, usually minimal, is found in 40% of cases. Medullary karyotype is always normal, without chromosome Ph1. Platelet functional abnormalities are not constant and do not correlate with the magnitude of thrombocythemia. 51 patients (84%) received myelosuppressive therapy, mainly by busulfan or radioactive phosphorus. Most deaths were due to intercurrent causes and only one patient developed acute leukemia. 71% of patients are alive at five years and subsequent decrease in the actuarial survival curve is very gradual.
本报告中描述的61例原发性血小板增多症病例约占作者在同一18年期间记录的真性红细胞增多症病例的15%。该组包括35名女性和26名男性，平均年龄为62岁。该病通常在常规血液检查时被发现（半数病例），因出血或血栓形成表现而被发现的情况较少。三分之一的病例有脾肿大。血小板计数持续高于800×10⁹/L（平均：1500×10⁹/L）；通常有轻度白细胞增多（平均：16×10⁹/L），以中性多形核白细胞为主，但骨髓白血病并不常见（28%的病例）且程度总是很轻；四分之三的病例红细胞参数正常，其余患者有贫血，原因可能是缺铁或其他因素。40%的病例有网状骨髓纤维化，通常很轻微。骨髓核型始终正常，无Ph1染色体。血小板功能异常并不常见，且与血小板增多的程度无关。51例患者（84%）接受了骨髓抑制治疗，主要是使用白消安或放射性磷。大多数死亡是由并发原因导致的，只有1例患者发展为急性白血病。71%的患者在5年后仍然存活，精算生存曲线随后的下降非常缓慢。
