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原发性骨淋巴瘤:临床表现与治疗考量

Primary bone lymphoma: Clinical presentation and therapeutic considerations.

作者信息

Müller Annika, Dreyling Martin, Roeder Falk, Baur-Melnyk Andrea, Knösel Thomas, Klein Alexander, Birkenmaier Christof, Jansson Volkmar, Dürr Hans Roland

机构信息

Musculoskeletal Oncology, Department of Orthopaedic Surgery, Physical Medicine and Rehabilitation, University Hospital, LMU Munich, Germany.

Department of Medicine III, University Hospital, LMU Munich, Germany.

出版信息

J Bone Oncol. 2020 Sep 28;25:100326. doi: 10.1016/j.jbo.2020.100326. eCollection 2020 Dec.

Abstract

BACKGROUND

Primary lymphoma of bone (PBL) is a rare entity. Due to unspecific clinical signs and equivocal radiographs diagnosis may be delayed. This retrospective report of 109 PBL cases demonstrates typical aspects of the lesion. Treatment and prognostic factors are evaluated.

METHODS

Retrospectively patient records were reviewed. All patients were followed for evidence of local or distant recurrence. Overall survival (OS) was used as clinical outcome.

RESULTS

The median age of the 109 patients was 62.8 years. The most common symptoms were pain (76%), swelling (29%), neurologic symptoms and pathological fracture (16% each). Mean duration of symptoms was 8 months (0-197 months).19% of patients had indolent NHL subtypes, 72% aggressive NHL subtypes and 7% cases Hodgkin disease. Cyclophosphamid, doxorubicin, vincristine and prednisone (CHOP) or CHOP plus rituximab (RCHOP) were given in 88 (81%) of patients. Radiotherapy was delivered in 67 (61%) of cases. 51 (47%) patients received both. Surgical interventions were restricted to cases with complications as fractures.The 5-year OS was 66%. The 5-year OS was 66%. In the subgroup of 78 patients with aggressive NHL subtype there was a highly significant benefit for chemotherapy or chemotherapy and radiation in comparison to no treatment or radiation alone. Raised LDH, age, IPI and ECOG performance were prognostic factors. In multivariate analysis, age and raised LDH levels only kept significance.

CONCLUSIONS

In our series of primary bone lymphoma, chemotherapy resulted in a better outcome than Radiotherapy alone. Long-term survival is based on the stage of the disease, favoring younger (<60 years) patients with solitary bone lesions, low level of LDH and favourable ECOG performance status and IPI scores.

摘要

背景

原发性骨淋巴瘤(PBL)是一种罕见疾病。由于临床症状不具特异性且X线表现不明确,诊断可能会延迟。这份对109例PBL病例的回顾性报告展示了该病变的典型特征。对治疗及预后因素进行了评估。

方法

对患者记录进行回顾性分析。所有患者均接受随访以观察局部或远处复发迹象。总生存期(OS)作为临床结局指标。

结果

109例患者的中位年龄为62.8岁。最常见症状为疼痛(76%)、肿胀(29%)、神经症状及病理性骨折(均为16%)。症状平均持续时间为8个月(0 - 197个月)。19%的患者为惰性非霍奇金淋巴瘤(NHL)亚型,72%为侵袭性NHL亚型,7%为霍奇金病。88例(81%)患者接受了环磷酰胺、阿霉素、长春新碱及泼尼松(CHOP)或CHOP加利妥昔单抗(RCHOP)治疗。67例(61%)患者接受了放疗。51例(47%)患者两者均接受。手术干预仅限于有骨折等并发症的病例。5年总生存率为66%。在78例侵袭性NHL亚型患者亚组中,与未治疗或单纯放疗相比,化疗或化疗联合放疗有显著益处。乳酸脱氢酶(LDH)升高、年龄、国际预后指数(IPI)及美国东部肿瘤协作组(ECOG)体能状态是预后因素。多因素分析显示,仅年龄和LDH水平升高仍具有统计学意义。

结论

在我们的原发性骨淋巴瘤系列研究中,化疗的效果优于单纯放疗。长期生存取决于疾病分期,更有利于年龄较轻(<60岁)、有孤立骨病变、LDH水平低、ECOG体能状态良好及IPI评分有利的患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ef7/7554647/dfaf17b65c29/gr1.jpg

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