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医学科普

欧洲移植工作组关于新冠疫情期间再生障碍性贫血患者诊治建议

发表者:何广胜 194人已读

APLASTIC ANEMIA AND CONSTITUTIONAL MARROW FAILURES江苏省人民医院血液内科何广胜


再生障碍性贫血和先天性骨髓衰竭


Some specificity related to aplastic anemia and constitutional marrow failure diseases are described below:

以下将描述与再生障碍性贫血和先天性骨髓衰竭相关的特征。

  1. Bone Marrow Transplantation (BMT).

骨髓移植

  1. It is recommended to postpone BMT whenever possible (according to disease severity, blood products immunization, infections risk) especially in case of unrelated transplantation, until the situation come back to normal, due to the risk of COVID-19 infection and also the possible lack of Intensive Care Unit availability, entirely occupied by severely COVID-19 injured patients.

建议根据疾病严重性,血制品免疫配型,感染风险尽可能延迟骨髓移植,尤其是无亲属关系移植,直到情况恢复到正常才进行。这是因为由于covid-19的感染以及因为严重的covid-19感染导致ICU病房床位的缺乏。

  1. BMT may be a saving treatment for severe aplastic anemia patients, and in several cases cannot be deferred. In case of urgent BMT, we recommend to carefully evaluate patients and donors for possible SARS-CoV-2 infection before admission in the hospital. A swab for SARS-CoV-2 should be performed before the start of the conditioning regimen and the patients should be admitted in BMT unit only once SARS-CoV-2 testing has proven negative (to avoid the risk of infecting the health care professionals). Transplant procedures (e.g., conditioning regimen, GVHD prophylaxis, post-transplant immunosuppression) do not need to be changed because of the SARS-CoV-2 outbreak.

骨髓移植对于重度的再障病人可能是挽救生命的治疗方法,(所以)在一些病例中不能被推迟。在一些紧急的骨髓移植术中,我们推荐在入院之前,仔细评估病人和捐赠者的SARS-CoV-2感染的可能性。在调理方案前,应该做一个SARS-CoV-2的咽拭子,直到仅仅当测试结果为阴性才能准许病人住进BMT的病房(为了避免感染医疗工作者的风险)。移植的步骤不需要因为SARS-CoV-2的爆发而改变(如调理方案,移植物抗宿主反应的预防,移植后免疫抑制)。

  1. Immunosuppressive therapy (applies only to acquired aplastic anemia):

免疫抑制疗法(仅仅用于获得性再障)

  1. There is no information about the risk of SARS-CoV-2 infection, nor about the clinical evolution of COVID-19, in patients who receive or have received immunosuppressive treatments. Nevertheless, it is commonly accepted that patients who have received intensive immunosuppression (i.e., using T-cell depleting agents such as horse-ATG, rabbit-ATG or Campath) have an increased morbidity and mortality from many viruses (including common coronaviruses). Thus, patients receiving T-cell depletion need to be considered at higher risk of SARS-CoV-2 infection.

在那些接受或者已经接受免疫抑制治疗的病人中,还没有关于SARS-CoV-2感染的数据,也没有临床COVID-19进化的数据。然而,普遍认为,那些接受强化免疫抑制治疗的病人(如使用像马ATG,ATG或者Campath之类的T细胞耗竭剂)对于很多病毒包括常见的冠状病毒的发病率和死亡率都增加了。所以接受T细胞耗竭剂治疗的病人需要考虑其对于SARS-CoV-2感染的高风险性。

  1. An intensive immunosuppressive therapy (IST) using T-cell depleting agents should be carefully considered in patients newly diagnosed with aplastic anemia. Treatment should be limited to patients with severe cytopenias with immediate risk of death (e.g., severe, or even very severe neutropenia), and patients who may safely stay at home. Theoretically, intensive IST might be appropriate in patients already hospitalized, but even here we should avoid the use of treatment who needs long-term hospitalization (anti- thymocyte globulin notably), to limit the presence of patients in the hospital and to free hospital resources which can be dedicated to COVID-19 emergency. As for BMT, we recommend to carefully evaluate patients for possible SARS-CoV-2 infection before admission in the hospital; ideally, they should be admitted in the unit only once SARS- CoV-2 testing has proven negative.

对于新诊断的再障的病人在使用强化的T细胞耗竭剂的免疫抑制治疗时应该认真考虑,该疗法应该仅限用于重度细胞减少有立即死亡风险的病人(如重度,甚至极重度嗜中性粒细胞减少症),对于待在家里能保持安全的病人可以延迟治疗。理论上,强化免疫抑制疗法可能适合于已经住院的病人,但是对于需要长期住院者,应该避免使用这种治疗方法(尤其是 抗人胸腺细胞球蛋白),从而减少这些病人的住院时间,为COVID-19感染的情况腾出医疗资源。对于骨髓移植治疗,在准许病人住院前,我们推荐去认真评估病人感染SARS-COV-2感染的可能性。理想情况下,他们仅仅当SARS-COV-2检测阴性时,才能住进病房。

  1. The use of alternative treatments which do not require hospitalization and do not lead to long-lasting immunodeficiency should be considered. In particular, the use of the

thrombopoietin-mimetic agent eltrombopag may be considered even as bridge to a more definitive treatment with BMT or intensive IST. This seems an acceptable compromise considering that for many patients the standard treatment of their aplastic anemia (either BMT or IST) is precluded by the COVID-emergency, and that in these circumstances eltrombopag treatment (even if not immediately effective) may reduce the immediate risk of complications associated with severe cytopenias.

不需要住院和可能导致长期持续免疫缺陷者应该考虑替代治疗。尤其是血小板激动剂艾曲泊帕,甚至可以被认为是骨髓移植和强化免疫抑制剂治疗的一种明确的桥接治疗。考虑到很多再障患者的标准治疗(骨髓移植或者免疫抑制治疗)被此次的冠状病毒紧急事件所阻止,这似乎是一种可以接受的折中治疗措施,而且在这些案例中,艾曲泊帕治疗方案(即使没有立即的效果)可能减少了重度全血细胞减少症的并发急性病症风险。

  1. For patients already receiving maintenance IST with cyclosporine (or other immunosuppressive agents requiring plasma level monitoring), no dose decrease should be applied during the following months to prevent the risk of relapse, in a future time in which hospital resources might be reduced because fully absorbed by COVID-19 infection. Furthermore, we recommend to keep maintenance doses within a level which do not require frequent monitoring (for drug plasma level, or even possible complications such as kidney failure), to limit the access to hospital or other medical structures for blood testings.

对于已经接受环孢素这种免疫抑制剂维持治疗(或者其他的需要监视血浆水平的免疫抑制剂),在接下来的数月里为了防止复发的风险,不要减少应用剂量。因为被covid-19感染者占用,医院的资源可能会减少。而且推荐把维持剂量保持在一定水平,在这个水平不需要频繁的检测血清浓度水平,甚至可能发生的肾衰竭,从而减少进入医院或者其他的医疗机构进行血液检测的机会。


  1. For relapsed patients, the risk-to-benefit assessment is the same as that of newly diagnosed aplastic anemia. In brief, keep patients at home whenever possibly, trying to defer intensive etiologic treatment with BMT or intensive IST. The early use of eltrombopag seems very reasonable in this situation.

对于复发的病人,和新诊断再障的病人一样需要进行风险效益评估。简而言之,尽可能让病人待在家里,推迟强化的病因学治疗(像骨髓移植和强化免疫抑制治疗)。在这种情况下,理论上值得早期使用艾曲泊帕。

In general whenever available, home care services have to massively utilized (and potentiated) both for eligible treatment delivery and for test monitoring (e.g. blood count, serum drug levels).

总之,只要有可能,应该大规模利用和强化家庭护理服务从而提供合适的治疗和检测(像血细胞计数,血清药物浓度水平)。


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发表于:2020-12-29 16:04

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