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[淀粉样轻链(AL)淀粉样变性与滤泡性淋巴瘤:一例报告]

[Amyloid light-chain (AL) amyloidosis and follicular lymphoma: a case report].

作者信息

Benmoussa Amine, Maatoui-Belabbes Hajar, Allali Reda, Regragui Meriem, Qachouh Meriem, Cherkaoui Siham, Lamchahab Mouna, Rachid Mohamed, Madani Abdellah, Khoubila Nisrine

机构信息

Service d'Hématologie Clinique, Faculté de Médecine et de Pharmacie Hassan II, Centre Hospitalier Universitaire Ibn Rochd Casablanca, Casablanca, Maroc.

Service d´Anatomie Pathologique, Faculté de Médecine et de Pharmacie Hassan II, Centre Hospitalier Universitaire Ibn Rochd Casablanca, Casablanca, Maroc.

出版信息

Pan Afr Med J. 2024 Dec 18;49:128. doi: 10.11604/pamj.2024.49.128.43592. eCollection 2024.

Abstract

The association of light-chain (AL) amyloidosis with type B follicular lymphoma is extremely rare, as the clone secreting the amyloidogenic light chain is generally plasmacytic. We here report the case of a 67-year-old patient with no specific pathological history, presenting with a deterioration in general condition, progressively worsening dysphonia and dysphagia, and a large mass in the cavum. Biopsy findings indicated grade 1-2 type-B follicular non-Hodgkin's lymphoma. Cervico-thoraco-abdomino-pelvic computed tomography (CT) scan revealed a nasopharyngeal mass measuring 70 mm x 40 mm and extending over 60 mm. Bone marrow biopsy and pre-therapeutic evaluations were normal. The patient received 4 cycles of rituximab plus CHOP (cyclophosphamide, adriamycine, prednisone and oncovin) with no response, followed by 3 cycles of rituximab plus DHAOX (dexamethasone, high-dose cytarabine and oxalipatin) with persistence of the mass. A subsequent biopsy of the mass revealed the disappearance of B-cell lymphoid infiltration but showed AL kappa chain amyloid deposits. Immunoelectrophoresis of plasma proteins detected the presence of IgM kappa immunoglobulin. Positron emission tomography (PET) imaging identified a hypermetabolic nasopharyngeal process. The patient is currently undergoing treatment with a protocol combining bortezomib, prednisone, and bendamustine.

摘要

轻链(AL)淀粉样变性与B型滤泡性淋巴瘤的关联极为罕见,因为分泌淀粉样轻链的克隆通常是浆细胞性的。我们在此报告一例67岁患者,无特殊病理病史,表现为全身状况恶化、进行性加重的声音嘶哑和吞咽困难,以及口腔内有一巨大肿块。活检结果显示为1-2级B型滤泡性非霍奇金淋巴瘤。颈胸腹部盆腔计算机断层扫描(CT)显示鼻咽部有一大小为70 mm×40 mm且延伸超过60 mm的肿块。骨髓活检和治疗前评估均正常。患者接受了4个周期的利妥昔单抗联合CHOP(环磷酰胺、阿霉素、泼尼松和长春新碱)治疗,但无反应,随后接受了3个周期的利妥昔单抗联合DHAOX(地塞米松、大剂量阿糖胞苷和奥沙利铂)治疗,肿块持续存在。随后对肿块进行活检,显示B细胞淋巴浸润消失,但可见AL κ链淀粉样沉积物。血浆蛋白免疫电泳检测到IgM κ免疫球蛋白的存在。正电子发射断层扫描(PET)成像显示鼻咽部有一高代谢病变。该患者目前正在接受硼替佐米、泼尼松和苯达莫司汀联合方案的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f61/11971929/0e646450c74c/PAMJ-49-128-g001.jpg

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