Wehmeier A, Daum I, Jamin H, Schneider W
Medizinische Klinik, Abteilung für Hämatologie, Düsseldorf, FRG.
Ann Hematol. 1991 Aug;63(2):101-6. doi: 10.1007/BF01707281.
Bleeding and thrombosis are frequent complications in myeloproliferative disorders (MPD) and are associated with severe organ damage and a high mortality. Elevated platelet count, elevated hematocrit, and patient age are regarded as risk factors for bleeding and thromboembolic events in MPD, although the significance of these parameters was not confirmed by clinical studies. We retrospectively analyzed vascular complications in 260 patients with MPD and tried to identify parameters predictive for bleeding and thrombembolic events. Our cohort consisted of 115 patients with chronic myeloid leukemia (CML), 84 patients with polycythemia vera (PV), 26 with essential thrombocythemia (ET), 25 with osteomyelofibrosis (OMF), and 10 patients with unclassifiable MPD. During a median follow-up period of 31 months, 126 patients with chronic MPD suffered bleeding or thrombotic events. Bleeding was observed in 57% of patients with OMF, 23% with PV, 20% with chronic phase CML, and 16% with ET. Thrombotic events were most common in patients with PV (36% of patients), followed by ET, OMF, and chronic phase CML (20%, 17%, and 6% of patients, respectively). Recurrent thrombotic episodes frequently occurred in patients with PV and ET, whereas patients with OMF often had more than one bleeding event. Thirty patients died of thrombohemorrhagic complications during follow-up. Multivariate analysis, including all patients with chronic MPD, revealed that elevated red blood cell count, higher hemoglobin level, and increased percentage of segmented neutrophils at the time of diagnosis were associated with thrombosis, whereas patients with bleeding complications were characterized by low red cell count, lower hemoglobin, and a lower percentage of segmented neutrophils. However, when analyzed by MPD subgroup, none of these parameters retained a predictive value for bleeding or thrombotic events. Moreover, elevated platelet count and patient age were not risk factors for bleeding complications. Thrombotic events were less frequent in patients below the age of 40, and were increased in patients aged 70 and above. However, this was primarily due to the high percentage of elderly patients in subgroups mainly affected by thrombosis (PV and ET). In most MPD subgroups, the rate of bleeding and thrombosis was highest just before and during the first months after diagnosis, and declined thereafter. Thrombohemorrhagic complications were less frequent after phlebotomy in PV and after therapy with alkylating agents in CML. The institution of cytoreductive therapy soon after the diagnosis was made may explain the reduced incidence of complications later in the disease. We conclude that morbidity and mortality from thrombohemorrhagic complications are high in myeloproliferative disorders.(ABSTRACT TRUNCATED AT 400 WORDS)
出血和血栓形成是骨髓增殖性疾病(MPD)常见的并发症,与严重的器官损害和高死亡率相关。血小板计数升高、血细胞比容升高和患者年龄被视为MPD出血和血栓栓塞事件的危险因素,尽管这些参数的意义尚未得到临床研究的证实。我们回顾性分析了260例MPD患者的血管并发症,并试图确定预测出血和血栓栓塞事件的参数。我们的队列包括115例慢性粒细胞白血病(CML)患者、84例真性红细胞增多症(PV)患者、26例原发性血小板增多症(ET)患者、25例骨髓纤维化(OMF)患者和10例无法分类的MPD患者。在中位随访期31个月期间,126例慢性MPD患者发生了出血或血栓形成事件。OMF患者中有57%发生出血,PV患者中为23%,慢性期CML患者中为20%,ET患者中为16%。血栓形成事件在PV患者中最常见(占患者的36%),其次是ET、OMF和慢性期CML(分别占患者的20%、17%和6%)。PV和ET患者经常发生复发性血栓形成发作,而OMF患者经常发生不止一次出血事件。30例患者在随访期间死于血栓出血并发症。对所有慢性MPD患者进行的多变量分析显示,诊断时红细胞计数升高、血红蛋白水平较高和分叶核中性粒细胞百分比增加与血栓形成相关,而出血并发症患者的特征是红细胞计数低、血红蛋白较低和分叶核中性粒细胞百分比较低。然而,按MPD亚组分析时,这些参数均未保留对出血或血栓形成事件的预测价值。此外,血小板计数升高和患者年龄不是出血并发症的危险因素。40岁以下患者血栓形成事件较少,70岁及以上患者则增加。然而,这主要是由于主要受血栓形成影响的亚组(PV和ET)中老年患者的比例较高。在大多数MPD亚组中,出血和血栓形成的发生率在诊断前及诊断后的头几个月最高,此后下降。PV患者放血后及CML患者接受烷化剂治疗后血栓出血并发症较少。诊断后不久开始进行细胞减灭治疗可能解释了疾病后期并发症发生率降低的原因。我们得出结论,骨髓增殖性疾病中血栓出血并发症的发病率和死亡率很高。(摘要截选至400字)