国际肿瘤学杂志

国际肿瘤学杂志 ›› 2019, Vol. 46 ›› Issue (10): 595-600.doi: 10.3760/cma.j.issn.1673-422X.2019.10.005

• 论著 • 上一篇    下一篇

Ph染色体/BCRABL融合基因阴性骨髓增殖性肿瘤患者不同疗效评价时期的症状负荷评估

章志福1,汤礼宾2,孙洪波1,刘军民1,罗伟1   

  1. 1深圳市龙华区人民医院血液科518109； 2深圳市龙华区人民医院产科518109

  • 出版日期:2019-10-08 发布日期:2019-12-20
  • 通讯作者: 章志福，Email： ygtgganhfw@163.com E-mail:ygtgganhfw@163.com
  • 基金资助:
    深圳市卫生计生系统科研项目（201607071）

Symptom load assessment of Ph chromosome/BCR-ABL fusion gene negative myeloproliferative tumor patients at different efficacy evaluation periods

Zhang Zhifu1, Tang Libin2, Sun Hongbo1, Liu Junmin1, Luo Wei1     

  1. 1Department of Hematology, People′s Hospital of Longhua District of Shenzhen, Shenzhen 518109, China; 2Department of Obstetric, People′s Hospital of Longhua District of Shenzhen, Shenzhen 518109, China 

  • Online:2019-10-08 Published:2019-12-20
  • Contact: Zhang Zhifu, Email: ygtgganhfw@163.com E-mail:ygtgganhfw@163.com
  • Supported by:
    Health Family Planning System Research Project of Shenzhen of China (201607071)

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摘要： 目的  观察Ph染色体/BCRABL融合基因阴性骨髓增殖性肿瘤（MPN）患者不同疾病类型及治疗过程中症状负荷的变化情况。方法  选取2017年5月至2019年1月深圳市龙华区人民医院确诊的84例MPN患者，根据其亚型分类将患者分为真性红细胞增多症（PV）组、原发性血小板增多症（ET）组、骨髓纤维化（PMF）组，每组各28例。比较3组患者骨髓增殖性肿瘤总症状评估量表（MPN-SAF-TSS）各维度评分情况，比较不同疗效时期（就诊时、疾病进展时、病情稳定时、临床改善时、部分缓解时、完全缓解时、复发时）肿瘤总症状评估量表各维度评分情况。结果  患者初诊时，PV组、ET组、PMF组MPN患者关于饱胀感（χ2=6.095，P=0.047）、腹部不适（χ2=7.342，P=0.025）、活动力不佳（χ2=13.029，P=0.001）、注意力不集中（χ2=6.099，P=0.047）、瘙痒（χ2=6.956，P=0.031）、骨痛（χ2=7.807，P=0.020）、发热（χ2=8.000，P=0.018）、体重减轻（χ2=27.340，P＜0.001）等症状负荷的发生率差异均具有统计学意义。其中PMF组患者活动力不佳（85.71%,24/28）、注意力不集中（67.86%,19/28）、体重减轻（82.14%,23/28）发生率显著高于PV组［42.86%(12/28)、39.29%(11/28)、35.71%(10/28)］、ET组［（46.43%(13/28)、39.29%(11/28)、14.29%(4/28)］（均P＜0.05），PMF组患者腹部不适（75.00%,21/28）、骨痛（60.71%,17/28）发生率高于PV组［39.29%(11/28）、25.00%(7/28)］（均P＜0.05）；PMF组饱胀感（89.29%,25/28）、发热（42.86%,12/28）发生率高于ET组［60.71%(17/28)、10.71%(3/28)］（均P＜0.05）；PV组瘙痒发生率（71.43%,20/28）高于ET组（42.86%,12/28）、PMF组（39.29%,11/28）（均P＜0.05）。在不同疗效时期，患者乏力（χ2=368.594，P<0.001）、饱胀感（χ2=261.312，P<0.001）、腹部不适（χ2=195.629，P<0.001）、活动力不佳（χ2=217.862，P<0.001）、注意力不集中（χ2=280.664，P<0.001）、夜间盗汗（χ2=239.650，P<0.001）、瘙痒（χ2=254.418，P<0.001）、骨痛（χ2=180.291，P<0.001）、发热（χ2=231.613，P<0.001）、体重减轻（χ2=227.831，P<0.001）的症状负荷评分，差异均具有统计学意义。其中疾病进展时乏力症状负荷评分较就诊时高（P<0.05），饱胀感症状负荷评分较就诊时低（P<0.05）；在病情稳定时乏力、瘙痒症状负荷评分较疾病进展时低（均P＜0.05）；在临床改善时乏力、腹部不适、注意力不集中、夜间盗汗、体重减轻症状负荷评分较疾病稳定时低（均P＜0.05）；在部分缓解时饱胀感、活动力不佳、注意力不集中、夜间盗汗、瘙痒症状负荷评分较临床改善时降低（均P<0.05）；在完全缓解时乏力、饱胀感、夜间盗汗、瘙痒、骨痛、体重减轻症状负荷评分较部分缓解时低（均P<0.05）；复发时乏力、饱胀感、腹部不适、活动力不佳、注意力不集中、夜间盗汗、瘙痒、骨痛、发热、体重减轻症状负荷评分较完全缓解时高（均P<0.05）。结论  各亚型MPN患者主要临床症状存在一定差别，随着病情进展或转归，其主要临床症状也有明显改变。

关键词:

骨髓肿瘤； 骨髓增殖性疾病； 真性红细胞增多症； 血小板增多, 原发性； 原发性骨髓纤维化； 骨髓增殖性肿瘤总症状评估量表

Abstract: Objective  To observe the disease type and the changes of symptom load during treatment of patients with Ph chromosome/BCR-ABL fusion gene negative myeloproliferative neoplasm (MPN). Methods  A total of 84 patients with MPN diagnosed from May 2017 to January 2019 in People′s Hospital of Longhua District of Shenzhen were selected, and were divided into polycythemia vera (PV) group, essential thrombocyhemia (ET) group, and myelofibrosis (PMF) group according to their subtypes, with 28 cases in each group. The scores of MPN-SAF-TSS were compared among the three groups. Besides, the scores of the scale (myeloproliferative neoplasm symptom assessment form total symptom score, MPN-SAF-TSS) in different treatment periods (at the time of the visit, when the disease progressed, when the disease was stable, when the clinical improvement was made, when the partial remission was completed, at the time of remission and recurrence) were also compared. Results  At the time of initial diagnosis, there were significant differences in the incidences of symptom burdens among the three groups of MPN patients with abdominal fullness (χ2=6.095, P=0.047), abdominal discomfort (χ2=7.342, P=0.025), poor mobility (χ2=13.029,P=0.001), inattention (χ2=6.099, P=0.047), pruritus (χ2=6.956,P=0.031), bone pain (χ2=7.807, P=0.020), fever (χ2=8.000, P=0.018) and weight loss (χ2=27.340, P＜0.001). The incidences of poor mobility (85.71%, 24/28), inattention (67.86%, 19/28) and weight loss (82.14%, 23/28) in PMF group were significantly higher than those in PV group ［42.86% (12/28), 39.29% (11/28), 35.71% (10/28)］  and ET group ［46.43% (13/28), 39.29% (11/28), 14.29% (4/28)］ (all P＜0.05). The incidences of abdominal discomfort (75.00%, 21/28) and bone pain (60.71%, 17/28) in PMF group were higher than those in PV group ［39.29% (11/28), 25.00% (7/28)］ (both P＜0.05). The incidences of   abdominal fullness (89.29%, 25/28)  and fever (42.86%, 12/28)  in PMF group were higher than those in ET group ［60.71% (17/28), 10.71% (3/28)］ (both P＜0.05). The incidence of pruritus in PV group (71.43%, 20/28) was higher than that in ET group (42.86%, 12/28) and PMF group (39.29%, 11/28) (both P＜0.05). Symptom load scores of patients with fatigue (χ2=368.594, P<0.001), abdominal fullness (χ2=261.312, P<0.001), abdominal discomfort (χ2=195.629, P<0.001), poor mobility (χ2=217.862, P<0.001), lack of concentration (χ2=280.664, P<0.001), night sweats (χ2=239.650, P<0.001), pruritus (χ2=254.418, P<0.001), bone pain (χ2=180.291, P<0.001), fever (χ2=231.613, P<0.001) and weight loss (χ2=227.831, P<0.001) were significantly different during different therapeutic periods. The fatigue symptom load score was higher when the disease progressed than that at the time of the visit (P<0.05), and the symptom score of abdominal fullness was lower than that at the time of visit (P<0.05). Symptom load scores of weakness and pruritus when the condition was stable was lower than those when the disease progressed (both P<0.05). When the clinical improvement was made, symptom load scores of weakness, abdominal discomfort, inattention, night sweats, weight loss were lower than those when the disease was stable (all P<0.05). Symptom load scores of abdominal fullness, poor mobility, inattention, night sweats and pruritus in partial remission period decreased compared to temporary improvement period (all P<0.05). Compared to the partial remission period, the symptom load scores of weakness, abdominal fullness, night sweats, pruritus, bone pain and weight loss in complete remission period were lower (all P<0.05). At last, symptom load scores of weakness, abdominal fullness, abdominal discomfort, poor mobility, inattention, night sweats, pruritus, bone pain, fever and weight loss in recurrence period were higher than those in complete remission period (all P<0.05). Conclusion  There are several differences in the main clinical symptoms among patients with different MPN subtypes, and there are significant changes in the main clinical symptoms as the disease progresses or turns around.

Key words: Bone marrow neoplasms, Myeloproliferative disorders, Polycythemia vera; Thrombocythemia, essential, Primary myelofibrosis, Myeloproliferative neoplasm symptom assessment form total symptom score