霍奇金淋巴瘤：近期進展回顧與展望
霍奇金淋巴瘤：近期進展回顧與展望
摘 要
霍奇金淋巴瘤（HL）是一種獨特的造血系統腫瘤，以炎性背景中的癌性Reed-Sternberg細胞為特徵。患者通常在20和30歲時被診斷患有HL，並且患有膈上淋巴結腫大，常伴有全身B癥狀。即使在晚期疾病中，HL在聯合化療，放療或聯合治療方案中也具有很高的治癒率。雖然同樣多柔比星，博來黴素，長春鹼和達卡巴嗪化療方案一直是治療的中流砥柱，在過去30年中，風險的適應方法已經幫助而對於風險較高的患者治療加劇去升級的低風險患者的治療。即使是初始治療未治癒的患者，也可以使用替代化療組合，新型抗體 - 藥物偶聯物brentuximab或高劑量自體或同種異體造血幹細胞移植進行搶救。程序性死亡1抑製劑nivolumab和pembrolizumab在複發/難治性HL患者中均表現出高響應率和持久緩解。 備用供體來源和降低強度的調節使異基因造血幹細胞移植成為更多患者的可行選擇。未來的研究將著眼於將新策略整合到早期治療方案中，以提高HL治癒率和減少長期治療毒性。
Hodgkin lymphoma (HL) is a unique hematopoietic neoplasm characterized by cancerous Reed-Sternberg cells in an inflammatory background. Patients are commonly diagnosed with HL in their 20s and 30s, and they present with supradiaphragmatic lymphadenopathy, often with systemic B symptoms. Even in advanced-stage disease, HL is highly curable with combination chemotherapy, radiation, or combined-modality treatment. Although the same doxorubicin, bleomycin, vinblastine, and dacarbazine chemotherapeutic regimen has been the mainstay of therapy over the last 30 years, risk-adapted approaches have helped de-escalate therapy in low-risk patients while intensifying treatment for higher risk patients. Even patients who are not cured with initial therapy can often be salvaged with alternate chemotherapy combinations, the novel antibody-drug conjugate brentuximab, or high-dose autologous or allogeneic hematopoietic stem cell transplantation. The programmed death-1 inhibitors nivolumab and pembrolizumab have both demonstrated high response rates and durable remissions in patients with relapsed/refractory HL. Alternate donor sources and reduced-intensity conditioning have made allogeneic hematopoietic stem cell transplantation a viable option for more patients. Future research will look to integrate novel strategies into earlier lines of therapy to improve the HL cure rate and minimize long-term treatment toxicities.
引 言
對於後來被稱為霍奇金病的第一個描述可以追溯到1832年，當時英國著名病理學家托馬斯霍奇金描述了一系列淋巴結病和脾腫大的屍檢病例。直到20世紀90年代後期，我們對實體作為由生髮中心或術後中心B細胞產生的惡性腫瘤導致術語霍奇金淋巴瘤（HL）獲得青睞。[2]典型地，癌細胞形成少數腫瘤並被包括淋巴細胞，嗜酸性粒細胞，嗜中性粒細胞，組織細胞和漿細胞。這些惡性細胞可以是特徵性的，多核巨細胞或大單核細胞，並且一起被稱為霍奇金和里德 - 斯騰伯格（HRS）細胞。
The first descriptions of what came to be known as Hodgkin disease date back to 1832, when the eminent British pathologist Thomas Hodgkin described an autopsy case series of patients with lymphadenopathy and splenic enlargement.1 It was not until the late 1990s that our understanding of the entity as a malignancy arising from germinal center or postgerminal center B cells led to the term Hodgkin lymphoma (HL) gaining favor.2 Characteristically, the cancer cells form a minority of the tumor and are surrounded by a reactive inflammatory milieu comprising lymphocytes, eosinophils, neutrophils, histiocytes, and plasma cells. These malignant cells can be pathognomonic, multinucleate giant cells or large mononuclear cells and, together, are referred to as Hodgkin and Reed-Sternberg (HRS) cells.
據估計，HL在美國新診斷的淋巴瘤病例中約佔10％（80,500例中有8260例），其餘為非霍奇金淋巴瘤（NHL）。由於淋巴瘤每年估計有21,210人死亡，約1070人（或5％）來自HL。它佔美國新診斷癌症病例的約0.5％，約佔所有癌症死亡的0.2％。然而，淋巴瘤是在青少年（15-19歲）診斷出的最常見的癌症，佔新診斷的21％，其中幾乎三分之二是HL。
It is estimated that HL accounts for approximately 10% of cases of newly diagnosed lymphoma in the United States (8260 of 80,500 cases), and the remainder are non-Hodgkin lymphoma (NHL). Of 21,210 estimated deaths yearly because of lymphoma, about 1070 (or 5%) are from HL. It accounts for about 0.5% of newly diagnosed cases of cancer in the United States and about 0.2% of all cancer deaths. However, lymphoma is the most common cancer diagnosed in adolescents (aged 15-19 years), accounting for 21% of new diagnoses, almost two-thirds of which are HL.
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