Differential Diagnosis of Waldenström's Macroglobulinemia and Early Management: Perspectives from Clinical Practice.

Shashank Cingam, Surbhi Sidana
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引用次数: 3

Abstract

Waldenström's Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM monoclonal protein and ≥10% bone marrow involvement with lymphoplasmacytic cells. Several mature B-cell and plasma cell disorders can potentially produce monoclonal IgM immunoglobulin and hence, careful consideration of the differential diagnosis is vital. Clinico-pathological features, immunophenotype, and MYD88 mutation status help distinguish WM from other plasma cell and lymphoproliferative disorders. Treatment is only indicated in patients symptomatic from adenopathy or organomegaly, neuropathy, hyper viscosity, cryoglobulinemia, cold agglutinin disease, cytopenia's or amyloidosis. Alkylators (cyclophosphamide, bendamustine) in combination with anti-CD20 antibodies and novel targeted agents including Bruton tyrosine kinase (BTK) inhibitors like ibrutinib are the mainstay of frontline treatment in symptomatic WM.

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Waldenström巨球蛋白血症的鉴别诊断和早期治疗:从临床实践的角度。
Waldenström's Macroglobulinemia (WM)是一种克隆性b淋巴细胞肿瘤,其特征是存在IgM单克隆蛋白和≥10%的骨髓浸润淋巴浆细胞。一些成熟的b细胞和浆细胞疾病可能产生单克隆IgM免疫球蛋白,因此,仔细考虑鉴别诊断是至关重要的。临床病理特征、免疫表型和MYD88突变状态有助于区分WM与其他浆细胞和淋巴细胞增生性疾病。治疗仅适用于有腺病或器官肿大、神经病变、高黏度、冷球蛋白血症、冷凝集素病、细胞减少症或淀粉样变性症状的患者。烷基化剂(环磷酰胺、苯达莫司汀)联合抗cd20抗体和新型靶向药物,包括布鲁顿酪氨酸激酶(BTK)抑制剂,如伊鲁替尼,是对症性WM一线治疗的主要方法。
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来源期刊
自引率
7.10%
发文量
16
审稿时长
16 weeks
期刊介绍: Blood and Lymphatic Cancer: Targets and Therapy is an international, peer reviewed, open access journal focusing on blood and lymphatic cancer research, identification of therapeutic targets, and the optimal use of preventative and integrated treatment interventions to achieve improved outcomes, enhanced survival, and quality of life for the cancer patient. Specific topics covered in the journal include: Epidemiology, detection and screening Cellular research and biomarkers Identification of biotargets and agents with novel mechanisms of action Optimal clinical use of existing anticancer agents, including combination therapies Radiation, surgery, bone marrow transplantation Palliative care Patient adherence, quality of life, satisfaction Health economic evaluations.
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