Kalantri Shreyas, Singh Inderpreet, Yang Long, Abuelgasim Khadega A
Internal Medicine, Sinai Hospital of Baltimore, Baltimore, MD, USA.
Pathology, Sinai Hospital of Baltimore, Baltimore, MD, USA.
Case Rep Oncol. 2023 May 23;16(1):351-356. doi: 10.1159/000530328. eCollection 2023 Jan-Dec.
Waldenstrom's macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by the infiltration of the bone marrow by clonal lymphoplasmacytic cells that produce monoclonal immunoglobulin M as defined by the World Health Organization Classification of hematological malignancies. Historically, the treatment options for WM were limited to alkylating agents and purine analogs. The introduction of immune therapy, including CD20 targeted therapy, proteasome inhibitors, and immune modulators, has provided benefit to those patients and has now become the standard of care. As WM patients become long-term survivors, treatment's late toxicities have become more apparent. Here, we report a case of a 74-year-old female who presented to the hospital with fatigue and was diagnosed with WM. She was treated with bortezomib, doxorubicin, and bendamustine, followed by rituximab. After a remission period of 15 years, the patient had a relapse of WM, and bone marrow biopsy findings were consistent with intermediate-risk t-MDS with complex cytogenetics, presenting us with a treatment dilemma. We decided to treat WM, and the patient went into VGPR with residual lymphoma cells. Despite having dysplasia and complex cytogenetics, she did not have any cytopenia. Currently, she is under observation anticipating the progression of her MDS, given her intermediate I risk status. This case features the occurrence of t-MDS after therapy with bendamustine, cladribine, and doxorubicin. This highlights the need for closer monitoring and consideration of long-term adverse effects when treating patients with indolent lymphomas, especially WM. Late complications need to be considered, and risk versus benefit analysis needs to be carefully evaluated, especially in younger patients with WM.
华氏巨球蛋白血症（WM）是一种淋巴浆细胞性淋巴瘤，其特征是骨髓被克隆性淋巴浆细胞浸润，这些细胞产生单克隆免疫球蛋白M，这是世界卫生组织血液系统恶性肿瘤分类所定义的。历史上，WM的治疗选择仅限于烷化剂和嘌呤类似物。免疫疗法的引入，包括CD20靶向疗法、蛋白酶体抑制剂和免疫调节剂，已使这些患者受益，现在已成为标准治疗方法。随着WM患者成为长期幸存者，治疗的晚期毒性变得更加明显。在此，我们报告一例74岁女性患者，她因疲劳入院，被诊断为WM。她接受了硼替佐米、阿霉素和苯达莫司汀治疗，随后接受利妥昔单抗治疗。在缓解期15年后，患者WM复发，骨髓活检结果与具有复杂细胞遗传学的中危治疗相关骨髓增生异常综合征（t-MDS）一致，给我们带来了治疗难题。我们决定治疗WM，患者达到了伴有残留淋巴瘤细胞的非常好的部分缓解（VGPR）。尽管存在发育异常和复杂的细胞遗传学，但她没有任何血细胞减少。鉴于她处于中危I风险状态，目前她正在接受观察，预期其MDS会进展。该病例的特点是在接受苯达莫司汀、克拉屈滨和阿霉素治疗后发生了t-MDS。这凸显了在治疗惰性淋巴瘤患者，尤其是WM患者时，需要更密切的监测并考虑长期不良反应。需要考虑晚期并发症，尤其在年轻的WM患者中，需要仔细评估风险与获益分析。
