Randi Maria Luigia, Fabris Fabrizio, Ruzzon Elisabetta, Pacquola Enrica, Cella Giuseppe, Girolami Antonio
Department of Medical and Surgical Sciences, University of Padua Medical School, Italy.
Haematologica. 2002 Nov;87(11):1180-4.
Polycythemia vera (PV) and essential thrombocythemia (ET) are two rare acquired myeloproliferative disorders (MPD) with frequent thrombotic and hemorrhagic complications. The occurrence of thrombosis in unusual sites, e.g. splanchnic vasculature, is a severe complication of these diseases. We describe a single-institution experience in patients with ET and PV, diagnosed in agreement with the Polycthemia Vera Study Group criteria, with portal vein thrombosis who did or did not undergo splenectomy.
The medical records and the follow-up outcome of 16 MPD patients with portal thrombosis who underwent splenectomy (group A1) and 16 who did not (group A2) were evaluated. Their median follow-up was, respectively, 13.45 and 10.49 years. The overall survival of these patients was compared with that of a population of 32 patients with MPD and no portal thrombosis (group B) matched for sex, age, diagnosis and duration of follow-up.
In group A1, 2 patients developed deep vein thrombosis, 1 patient had a surgical hemorrhage and 2 patients died early, one from acute infection, the other from bone marrow aplasia. Among the survivors, one male had a deep vein thrombosis and 1 developed a new portal thrombosis. Four patients died during the follow-up (median 9.48 years, range 3.17-25.1; 1 stroke, 2 gastrointestinal bleedings, 1 leukemic conversion). No difference was observed in the incidence of thrombotic or hemorrhagic complications or in the rate of deaths when group A1 was compared to the other groups. The use of antiplatelets drugs was statistically increased in group A1 after splenectomy, because portal vein thrombosis induced per se an increased use of therapeutic agents. No statistical difference was observed in overall survival between the different groups.
真性红细胞增多症（PV）和原发性血小板增多症（ET）是两种罕见的获得性骨髓增殖性疾病（MPD），常伴有血栓形成和出血并发症。在不常见部位发生血栓，如内脏血管，是这些疾病的严重并发症。我们描述了在一家机构中，依据真性红细胞增多症研究组标准诊断为ET和PV且伴有门静脉血栓形成的患者，接受或未接受脾切除术的情况。
对16例接受脾切除术的MPD门静脉血栓形成患者（A1组）和16例未接受脾切除术的患者（A2组）的病历及随访结果进行评估。他们的中位随访时间分别为13.45年和10.49年。将这些患者的总生存率与32例MPD且无门静脉血栓形成的患者（B组）进行比较，B组患者在性别、年龄、诊断及随访时间上相匹配。
在A1组中，2例患者发生深静脉血栓形成，1例患者出现手术出血，2例患者早期死亡，1例死于急性感染，另1例死于骨髓再生障碍。在幸存者中，1例男性发生深静脉血栓形成，1例出现新的门静脉血栓形成。4例患者在随访期间死亡（中位时间9.48年，范围3.17 - 25.1年；1例中风，2例胃肠道出血，1例白血病转化）。将A1组与其他组比较，在血栓形成或出血并发症的发生率及死亡率方面未观察到差异。脾切除术后A1组抗血小板药物的使用在统计学上有所增加，因为门静脉血栓形成本身导致治疗药物使用增加。不同组之间在总生存率方面未观察到统计学差异。
1）出血和血栓形成是伴有或不伴有脾切除术的ET和PV门静脉血栓形成患者发病和死亡的主要原因。2）门静脉血栓形成，有时还有脾切除术，需要增加药物使用，这可能会增加白血病转化的风险。尽管如此，在脾切除术后第一个阶段存活下来的患者可能会有漫长且安全的生存期。
