A Case of Recurrent Localized Pulmonary Nodular Light Chain Amyloidosis Treated with Daratumumab plus CyBorD.

IF 1.7 4区医学 Q3 HEMATOLOGY

Acta Haematologica Pub Date : 2024-07-29 DOI:10.1159/000540272

Ted Raddell, Farah Ashraf, Xiaofeng Zhao, Osheen Abramian, Tulin Budak-Alpdogan

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引用次数: 0

Abstract

Introduction: Nodular pulmonary amyloidosis (NPA) is a localized form of light chain (AL) amyloidosis often found incidentally and typically has an indolent and benign disease course treated with resection or local excision. We present a patient with recurrent localized AL amyloidosis who required further treatment.

Case presentation: A 63-year-old female with monoclonal gammopathy of undetermined significance (MGUS) was found to have pulmonary AL amyloid on wedge resection and later had recurrence. The patient did not have signs of clonal plasma cell proliferation or systemic AL amyloid. She was treated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone. After initiation of treatment, the patient has had significant hematologic and radiographic response.

Conclusion: The patient had NPA recurrence with organ dysfunction without systemic disease. Because the presentation of recurrent pulmonary AL amyloidosis is rare, there is no published evidence on treatment. However, the patient has had hematologic and radiographic improvement after initiating treatment with a systemic protocol.

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达拉单抗加 CyBorD 治疗一例复发性局部肺结节性 AL 淀粉样变性病例

导读：结节性肺淀粉样变性（NPA）是一种局部型AL淀粉样变性，通常是偶然发现的，其病程通常是不活跃的良性病程，治疗方法是切除或局部切除。我们为您介绍一位需要进一步治疗的复发性局部 AL 淀粉样变性患者。病例描述一位患有 MGUS 的 63 岁女性患者在接受楔形切除术时被发现患有肺 AL 淀粉样变性，后来又复发了。患者没有克隆性浆细胞增生或全身性 AL 淀粉样变性的迹象。她接受了达拉单抗、环磷酰胺、硼替佐米和地塞米松治疗。治疗开始后，患者的血液学和影像学反应都很明显。结论患者为结节性肺淀粉样变性复发，伴有器官功能障碍，无全身性疾病。由于复发性肺AL淀粉样变性的表现非常罕见，目前还没有关于治疗的公开证据。不过，在开始接受系统方案治疗后，患者的血液学和影像学状况有所改善。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta Haematologica 医学-血液学

CiteScore

4.90

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： ''Acta Haematologica'' is a well-established and internationally recognized clinically-oriented journal featuring balanced, wide-ranging coverage of current hematology research. A wealth of information on such problems as anemia, leukemia, lymphoma, multiple myeloma, hereditary disorders, blood coagulation, growth factors, hematopoiesis and differentiation is contained in first-rate basic and clinical papers some of which are accompanied by editorial comments by eminent experts. These are supplemented by short state-of-the-art communications, reviews and correspondence as well as occasional special issues devoted to ‘hot topics’ in hematology. These will keep the practicing hematologist well informed of the new developments in the field.