Division of Hematology, Mayo Clinic, Rochester, MN.
Am J Hematol. 2014 Jul;89(7):771-9. doi: 10.1002/ajh.23750.
DISEASE OVERVIEW: Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 9,200 new patients annually and representing approximately 11.5% of all lymphomas in the United States. DIAGNOSIS: HL is composed of two distinct disease entities; the more commonly diagnosed classical HL and the rare nodular lymphocyte-predominant HL. Nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich HL are subgroups under the designation of classical HL. RISK STRATIFICATION: An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy. Prognostic models that identify patients at low or high risk for recurrence, as well as the response to therapy as determined by positron emission tomography scan, are used to optimize therapy. RISK-ADAPTED THERAPY: Initial therapy for HL patients is based on the histology of the disease, the anatomical stage, and the presence of poor prognostic features. Patients with early stage disease are treated with combined modality strategies using abbreviated courses of combination chemotherapy followed by involved-field radiation therapy, while those with advanced stage disease receive a longer course of chemotherapy often without radiation therapy. Management of relapsed/refractory disease: High-dose chemotherapy (HDCT) followed by an autologous stem cell transplant (ASCT) is the standard of care for most patients who relapse following initial therapy. For patients who fail HDCT with ASCT, brentuximab vedotin, palliative chemotherapy, nonmyeloablative allogeneic transplant, or participation in a clinical trial should be considered.
疾病概述:霍奇金淋巴瘤(HL)是一种罕见的 B 细胞淋巴瘤,每年影响 9200 名新患者,约占美国所有淋巴瘤的 11.5%。
诊断:HL 由两种不同的疾病实体组成;更常见的诊断为经典 HL 和罕见的结节性淋巴细胞为主型 HL。结节性硬化、混合细胞性、淋巴细胞耗竭和富含淋巴细胞的 HL 是经典 HL 下的亚组。
风险分层:准确评估 HL 患者的疾病分期对于选择适当的治疗至关重要。用于识别复发风险低或高的患者以及通过正电子发射断层扫描确定对治疗的反应的预后模型,用于优化治疗。
风险适应治疗:HL 患者的初始治疗基于疾病的组织学、解剖分期和不良预后特征的存在。早期疾病患者采用联合模式策略,使用缩短疗程的联合化疗,然后进行受累野放疗,而晚期疾病患者则接受更长疗程的化疗,通常不进行放疗。复发/难治性疾病的治疗管理:对于大多数初始治疗后复发的患者,高剂量化疗(HDCT)后自体干细胞移植(ASCT)是标准治疗。对于接受 ASCT 后 HDCT 失败的患者,应考虑使用 Brentuximab Vedotin、姑息性化疗、非清髓性同种异体移植或参加临床试验。
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