多发性骨髓瘤的罕见表现:胸腔积液和广泛髓外受累

Kiran Pk, G Hari Prakash, Sahana Kr, Sunil Kumar D
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摘要

60岁女性,腹痛,体重减轻,乏力。影像学显示胰腺肿块,双侧胸腔积液,腹水和溶解性骨病变。调查证实多发性骨髓瘤伴轻链病。正电子发射断层扫描-计算机断层扫描(PET-CT)显示广泛的代谢性软组织肿块,包括胰腺区、腹膜后、纵隔、椎旁区域,以及多发性骨外软组织受累的骨骼病变,以及双侧胸膜积液,伴代谢性胸膜和胸膜外沉积物。患者开始使用硼替佐米、环磷酰胺和地塞米松化疗方案,并进行胸腔穿刺治疗以治疗胸腔积液。两个周期后,患者临床表现明显改善。重复PET-CT扫描显示软组织肿块明显间隔消退,代谢活动消退,胸膜和胸膜外沉积物消退。广泛的骨溶解病变表现为形态学稳定,但相关的代谢活性和骨外软组织退化。该病例强调了新型药物治疗方案在多发性骨髓瘤伴广泛性髓外疾病(EMD)(包括胸腔积液等不常见症状)患者中取得特殊疗效的潜力。早期识别和及时开始适当的治疗对于改善这类病例的结果至关重要。
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Uncommon Presentation of Multiple Myeloma: Pleural Effusion and Extensive Extramedullary Involvement.

A 60-year-old female presented with abdominal pain, weight loss, and fatigue. Imaging revealed a pancreatic mass, bilateral pleural effusion, ascites, and lytic bony lesions. Investigations confirmed multiple myeloma with lambda light chain disease. Positron emission tomography-computed tomography (PET-CT) scan demonstrated extensive metabolically active soft tissue masses involving the pancreatic region, retroperitoneum, mediastinum, paravertebral regions, and multiple skeletal lesions with extraosseous soft tissue involvement, along with bilateral pleural effusions with metabolically active pleural and extrapleural deposits. The patient was initiated on a bortezomib, cyclophosphamide, and dexamethasone chemotherapy regimen with therapeutic thoracentesis for pleural effusion management. After two cycles, the patient showed remarkable clinical improvement. A repeat PET-CT scan revealed significant interval regression of soft tissue masses, metabolic activity resolution, and regression of pleural and extrapleural deposits. The extensive skeletal lytic lesions showed morphological stability but regression of associated metabolic activity and extraosseous soft tissue. This case highlights the potential of novel agent-based regimens in achieving exceptional responses in multiple myeloma patients with extensive extramedullary disease (EMD), including uncommon manifestations like pleural effusion. Early recognition and prompt initiation of appropriate therapy are crucial for improving outcomes in such cases.

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