外周T细胞淋巴瘤的事实.doc

外周T细胞淋巴瘤的事实
No. 25 in a series providing the latest information for patients, caregivers and healthcare professionals 系列中的第25号患者，医护人员以及医疗专业人员提供最新信息
Highlights 亮点
• Peripheral T-cell lymphomas (PTCLs) comprise a diverse group of uncommon and aggressive diseases •外周T细胞淋巴瘤（PTCLs）包括一个罕见的和积极的疾病不同群体
in which the patient's T cells become cancerous. 在病人的T细胞发生癌变。 T-cell lymphomas account for between 10 percent and T细胞淋巴瘤占10％至
15 percent of all non-Hodgkin lymphomas (NHLs). 15％的所有非霍奇金淋巴瘤（NHLs）。
• The World Health Organization (WHO) divides PTCLs into three categories, nodal, extranodal and 世界卫生组织（WHO）的划分为三类PTCLs，淋巴结，结外和
leukemic, and has classified 13 major subtypes of PTCLs. 白血病，并已列为13 PTCLs主要亚型。 Getting an accurate diagnosis and knowing 获取准确的诊断和了解
your PTCL subtype is important. 您的PTCL的亚型是很重要的 。
• PTCLs are rare in the United States and are more common in Asia, Africa and the Caribbean, possibly •PTCLs在美国是罕见的，并在亚洲，非洲和加勒比地区，可能更常见
due to exposure to specific viruses, such as the Epstein-Barr virus (EBV) and the human T-cell 由于暴露于特定的病毒，如的EB病毒（EBV）和人类T细胞，
leukemia virus-1 (HTLV-1). 白血病病毒1型（HTLV - 1）。
• PTCLs generally affect people older than 60 years, although they can occur anytime during adulthood. •PTCLs一般影响60岁以上的人，虽然他们可以随时发生在成年期。
• Although the signs and symptoms of PTCLs vary according to the subtype, some common signs and •虽然PTCLs的迹象和症状不同亚型，一些常见的体征
symptoms of the diseases include fatigue, a painless swelling in the neck, armpit or groin (due to an 疾病的症状包括疲劳，在颈部，腋窝或腹股沟的无痛性肿大 （由于
enlarged lymph node), night sweats, rash and weight loss. 扩大淋巴结），盗汗，皮疹和减肥。
• New therapies are showing some effectiveness in treating patients who have certain subtypes of PTCL, •新疗法在治疗PTCL的某些亚型的患者一些成效 ，
and other potential therapies are being studied in clinical trials. 和其他潜在疗法的临床试验正在研究。 However, standards of care have not 然而，护理标准
been established for newly diagnosed PTCL patients or for patients who have disease that has relapsed 已建立新诊断PTCL的患者或患者有疾病的人有复发的
(recurred) or is refractory (resistant to treatment). （复发）或难治性（耐治疗）。
• More research and clinical trials focusing specifically on the various subtypes of PTCL are needed to •更多的研究和临床试验，特别侧重于PTCL的各种亚型都需要
define the optimal management of patients who have these diseases. 定义有这些疾病的患者的最佳管理。
Introduction 简介
Peripheral T-cell lymphomas (PTCLs) are uncommon and aggressive non-Hodgkin lymphomas (NHLs) 外周T细胞淋巴瘤（PTCLs）是罕见的侵袭性非霍奇金淋巴瘤（NHLs）
that develop in mature white blood cells called “T cells” and “natural killer (NK) cells.” NHL is the ，发展成熟的白 ​​细胞称为“T细胞”和“自然杀伤（NK）细胞。”细胞NHL是
name for many different types of cancer that start in cells called “lymphocytes,” a type of white cell that 名称为许多不同类型的癌症，细胞开始被称为“淋巴细胞，白细胞类型
helps the body fight infection. 帮助人体抵抗感染。 There are two main types of lymphocytes: B lymphocytes (B cells) and 主要有两种类型的细胞：B淋巴细胞（B细胞）和
T lymphocytes (T cells). T淋巴细胞（T细胞）。 NHL may arise in B cells or T cells. 国家冰球联赛中可能出现的B细胞或T细胞。 B-cell lymphomas are more common than B细胞淋巴瘤是较常见的比
T-cell lymphomas. T细胞淋巴瘤。 NHLs may be indolent (slow growing) or aggressive (fast growing). NHLs可好逸恶劳（生长缓慢）或侵略（快速增长）。

Page 2 第2页
The Leukemia & Lymphoma Society • 1311 Mamaroneck Avenue • White Plains, NY 10605 白血病和淋巴瘤协会 •1311马马罗内克大道•白色平原，纽约10605
Information Specialist 800.955.4572 • www.LLS.org 信息专家800.955.4572•www.LLS.org
Peripheral T-Cell Lymphoma Facts/2 外周T细胞淋巴瘤的事实/ 2
For additional information about NHL, please see the free Leukemia & Lymphoma Society (LLS) 有关国家冰球联赛的其他信息，请参阅免费的白血病和淋巴瘤协会（LLS ）
booklets Non-Hodgkin Lymphoma and The Lymphoma Guide – Information for Patients and 小册子 非霍奇金淋巴瘤和淋巴瘤指南-患者信息
Caregivers . 护理人员 。
This publication provides descriptions of the various subtypes of PTCL. 本刊物提供PTCL的各种亚型的描述 。 It also includes specific 这也包括具体的
information on the diagnosis, stages and treatment of PTCLs; new drugs being studied in clinical trials; 信息的诊断，分期和治疗PTCLs;在临床试验正在研究新的药物;
and support resources. 和支持资源。
About Peripheral T-Cell Lymphoma 关于外周T细胞淋巴瘤
Between 10 percent and 15 percent of all patients with NHL have a T-cell lymphoma subtype. 非霍奇金淋巴瘤的所有患者的10％和15％之间的有T细胞淋巴瘤亚型。 PTCLs PTCLs
generally affect people aged 60 years and older and are diagnosed slightly more often in men than in 一般影响年龄在60岁及以上的人略多往往比男性被确诊
women. 妇女。 However, younger adults and children are also diagnosed with PTCLs. 然而，年轻的成年人和儿童，也诊断PTCLs 。 PTCL is an uncommon PTCL的是一种罕见的
disease in the United States. 在美国疾病。 Some forms of PTCL are more common in Asia, Africa and the Caribbean, PTCL的一些形式较为常见，在亚洲，非洲和加勒比地区 ，
possibly as a result of exposure to specific viruses, such as the Epstein-Barr virus (EBV) and the human 可能是由于暴露于特定的病毒，如爱泼斯坦-巴尔病毒（EBV）和人类的结果
T-cell leukemia virus-1 (HTLV-1). T细胞白血病病毒1型（HTLV - 1）。
The World Health Organization (WHO) classification system recognizes 13 different subtypes of PTCL 世界卫生组织（WHO）的分类系统承认的PTCL的13个不同的亚型
and has grouped the diseases into three categories: nodal, extranodal and leukemic. 分组分为三类疾病：淋巴结，结外病变和白血病。 WHO has also 世卫组织还
divided T-cell lymphomas into two groups: aggressive (fast growing) and indolent (slow growing). T细胞淋巴瘤分为两组：进取 ​​（快速增长）和懒惰（生长缓慢）。
PTCLs are a varied group of diseases that differ from B-cell lymphomas. PTCLs是从B细胞淋巴瘤不同的疾病，不同的组。 Because PTCLs are less 由于PTCLs少
common than B-cell lymphomas, they have not been as well understood. 比B细胞淋巴瘤的共同，他们并没有被很好地理解 。 Techniques to distinguish and 技术区分和
study the various subtypes of PTCL have only recently been developed. 研究PTCL的各种亚型，只有最近被开发 。 As a result, standards of care for 因此，护理标准
how best to treat PTCLs have not been established for newly diagnosed patients or for patients whose 如何最好的治疗PTCLs尚未确定为初诊患者或患者的
disease has relapsed (recurred) or is refractory (resistant to treatment). 病有复发（复发）或难治性（耐治疗）。 In general, treatment outcomes 在一般情况下，治疗结果
have been poor with conventional chemotherapy regimens. 与传统的化疗方案已差 。 However, a greater understanding of PTCLs 然而，更深入的了解，PTCLs
and new genetic and molecular testing techniques has led to the development of new targeted drugs 和新的遗传和分子检测技术，导致新的靶向药物的发展
(see Treatment Under Study on page 5). （见第5页上正在研究的治疗） 。 Other therapies are being explored and tested in research其他疗法正在探索和研究测试
laboratories and in human clinical trials designed specifically for T-cell lymphomas (see Peripheral 实验室和专门设计的T细胞淋巴瘤的人体临床试验（ 见外设
T-Cell Lymphoma Subtypes on pages 7 through 8).第7页8）T细胞淋巴瘤亚型 。
Because PTCLs are so uncommon, it is best to seek treatment at a medical center specializing in the 因为PTCLs如此少见，最好是在一个医疗中心寻求治疗
diagnosis and treatment of NHL (see the free LLS fact sheet Choosing a Blood Cancer Specialist or 非霍奇金淋巴瘤的诊断和治疗（见自由LLS的事实 表选择血液肿瘤专科或
Treatment Center for more information). 治疗中心了解更多信息）。
One group of T-cell lymphomas is called “cutaneous T-cell lymphomas (CTCLs),” or skin lymphomas. 有一组T细胞淋巴瘤被称为“皮肤T细胞淋巴瘤（CTCLs）”，或皮肤淋巴瘤 。
CTCLs consist of a number of different diseases with various signs and symptoms, treatment approaches CTCLs各种症状和体征，治疗方法包括了许多不同的疾病
and outcomes. 和成果。 While there may be skin involvement with some PTCLs, CTCLs originate in the skin. 虽然有可能是皮肤与一些PTCLs参与，CTCLs起源于皮肤。
CTCLs are primarily slow growing. CTCLs主要是生长缓慢。 This group of diseases is described in detail in the free LLS fact sheet 这组疾病是在免费的LLS的事实表中详细描述
Cutaneous T-Cell Lymphoma . 皮肤T细胞淋巴瘤 。

Page 3 第3页
The Leukemia & Lymphoma Society • 1311 Mamaroneck Avenue • White Plains, NY 10605 白血病和淋巴瘤协会 •1311马马罗内克大道•白色平原，纽约10605
Information Specialist 800.955.4572 • www.LLS.org 信息专家800.955.4572•www.LLS.org
Peripheral T-Cell Lymphoma Facts/3 外周T细胞淋巴瘤的事实/ 3
Signs and Symptoms 症状和体征
The first signs of PTCL vary depending on the disease subtype. 取决于疾病亚型的PTCL的第一个迹象。 Because lymph nodes in several different 因为在几个不同的淋巴结
areas of the body are frequently involved, the most common sign of PTCL is an enlarged, painless lymph 身体的地区经常参与，最常见的PTCL的标志是一个扩大的，无痛性淋巴结
node in the neck, armpit or groin. 在颈部，腋窝或腹股沟的节点 。 Enlarged lymph nodes can appear near the ears or elbows. 肿大的淋巴结可出现耳朵或手肘附近。 These 这些
lymphomas also affect various organs in the body, including the bone marrow, liver, spleen, stomach and 淋巴瘤也影响到身体的各个器官，包括骨髓，肝，脾，胃和，
skin. 皮肤。 Other symptoms may include 其他症状可能包括
• Night sweats •盗汗
• Fever •发热
• Weight loss •减肥
• Rash. •皮疹。
Diagnosis 诊断
Most PTCLs are diagnosed by taking a small sample (a “biopsy”) of an enlarged lymph node and then 大多数PTCLs通过采取扩大淋巴结的一个小样本（“活检”），然后被确诊
examining the cells under a microscope. 检查在显微镜下的细胞。 Generally, either the lymph node, or a part of the lymph node, is 一般情况下，无论是淋巴结，或淋巴结的一部分，是
surgically removed so that the hematopathologist (a doctor who specializes in interpreting and diagnosing 手术切除，使hematopathologist（一个专门的解释和诊断的医生
the physical changes caused by diseases of the blood and marrow) has enough tissue to make a firm 的血液和骨髓疾病引起的物理变化）有足够的组织作出坚定的
diagnosis. 诊断。 Lymph node biopsy tissue can often be removed after the administration of a local anesthetic. 淋巴结活检组织通常可消除局部麻醉后的管理 。
The cells in many subtypes of PTCLs look alike; therefore, making an accurate diagnosis may require PTCLs许多亚型的细胞看起来很像，因此，作出准确的诊断，可能需要
the use of additional diagnostic tests, including blood tests; CT (computerized axial tomography), PET 使用额外的诊断测试，包括验血，CT（电脑断层）， PET
(positron emission tomography) scans and MRI (magnetic resonance imaging); and a bone marrow biopsy. （正电子发射断层扫描）扫描和MRI（磁共振成像）;和骨髓活检。
An accurate diagnosis is the basis for planning the treatment approach. 准确的诊断是规划的治疗方法的基础上。 An experienced hematopathologist 一个经验丰富的hematopathologist
is needed to analyze the biopsy slides. 需要分析的活检幻灯片。 A second opinion by another hematopathologist may be necessary 一个由另一hematopathologist第二意见可能有必要
if there is any doubt about the diagnosis. 如果有任何怀疑诊断。
Treatment Planning 治疗计划
The goal of treatment for many aggressive lymphomas is a cure. 许多侵袭性淋巴瘤的治疗目标是治愈 。 Every patient's medical situation is 每一个病人的医疗状况
different and should be evaluated individually by an oncologist who specializes in treating NHL. 不同的，应单独计算，由专门治疗NHL的肿瘤学家 。 It 它
is important for you and members of your oncology team to discuss all treatment options, including 重要的是，您和您的肿瘤团队成员讨论所有的治疗方案，包括
treatments being studied in clinical trials. 治疗正在研究在临床试验中。
Staging 分期
Knowing the stage of your disease helps members of your medical team determine the most effective 了解你的病的阶段，有助于您的医疗团队成员确定最有效的
course of treatment for you. 当然，对你的治疗 。 The Ann Arbor Staging System is the most common system used for classifying Ann Arbor分期系统是最常见的系统进行分类
all subtypes of NHL. 非霍奇金淋巴瘤的所有亚型 。

Page 4 第4页
The Leukemia & Lymphoma Society • 1311 Mamaroneck Avenue • White Plains, NY 10605 白血病和淋巴瘤协会 •1311马马罗内克大道•白色平原，纽约10605
Information Specialist 800.955.4572 • www.LLS.org 信息专家800.955.4572•www.LLS.org
Peripheral T-Cell Lymphoma Facts/4 外周T细胞淋巴瘤的事实/ 4
The system is divided into four stages and is based on where the disease is located in the body: 该系统分为四个阶段，并基于这种疾病是位于身体 ：
• Stage I—The cancer is in a single lymph node or lymph node region, for example, above or below •第一阶段的癌症是在一个单一的淋巴结或淋巴结地区的，例如，高于或低于
the diaphragm; or, the cancer is in an organ or site other than a lymph node (extranodal) but has 隔膜;，癌症是一个器官或淋巴结（结外）以外的网站，但已
not spread to other organs or lymph nodes. 不会蔓延到其他器官或淋巴结。
• Stage II—The cancer is in two or more lymph node regions on the same side of the diaphragm; •第二阶段的癌症是在两个或两个以上的淋巴结节点地区，同方的隔膜 ;
or, the disease has spread to one or more lymph node groups on the same side of the diaphragm. 或疾病已经蔓延到一个或多个淋巴结组，同方的隔膜 。
• Stage III—The cancer is in lymph node regions on both sides of the diaphragm, with or without •第三阶段的癌症是在隔膜两侧的淋巴结区，带或不带
partial involvement of an extranodal organ or site above or below the diaphragm. 部分参与的一个结外器官或高于或低于隔膜网站。
• Stage IV—The cancer is widespread, including multiple involvements in one or more extranodal •第四阶段的癌症是广泛的，包括在一个或更多的结外多个介入
sites, such as the bone marrow. 网站，如骨髓。
Treatment for Newly Diagnosed Patients 初诊患者的治疗
Currently, newly diagnosed PTCL patients are usually treated with anthracycline-based chemotherapy 目前，新确诊的PTCL的患者通常是治疗蒽环类药物为主的化疗
regimens. 方案。 Most subtypes of PTCL are treated as follows: PTCL的大多数亚型如下处理：
• CHOP (cyclophosphamide, hydroxydoxorubicin [doxorubicin], Oncovin •用CHOP（环磷酰胺，hydroxydoxorubicin [阿霉素，Oncovin
® ®
[vincristine], prednisone) [长春新碱，强的松）
• EPOCH (etoposide, prednisone, Oncovin •EPOCH（依托泊苷，强的松，Oncovin
® ®
[vincristine], cyclophosphamide, hydroxydoxorubicin [长春新碱，环磷酰胺，hydroxydoxorubicin
[doxorubicin] ) [阿霉素]）
• Hyper-CVAD (cyclophosphamide, Oncovin •超- CVAD（环磷酰胺，Oncovin
® ®
[vincristine], Adriamycin [长春新碱，阿霉素
® ®

[doxorubicin], [阿霉素]
dexamethasone); “hyper” refers to “hyperfractionation of the dose,” meaning that the 地塞米松），“超”是指“剂量超分割”，意思是，
chemotherapy is given in small, frequent doses to minimize side effects. 小，频繁的剂量化疗，以尽量减少副作用 。
• Clinical trials with new combinations of chemotherapies. •新组合化疗的临床试验 。
Unfortunately, cure rates for PTCL remain low with the exception of the cure rates for 不幸的是，治疗PTCL的利率仍然异常治愈率为低
• ALK-positive anaplastic large cell lymphoma (see Peripheral T-Cell Lymphoma Subtypes •ALK阳性间变性大细胞淋巴瘤（见 外周T细胞淋巴瘤亚型
on pages 7 through 8) 7至8页）
• Localized extranodal NK/T-cell lymphoma, for which localized radiotherapy and anthracycline- •本地化的结外NK / T细胞淋巴瘤，局部放疗和蒽环类
based chemotherapy are usually recommended. 通常建议为基础的化疗。
Patients with PTCL should consult with the members of their medical team about the availability of 与PTCL的患者应咨询他们的医疗团队成员的可用性
appropriate clinical trials for initial treatment (see Treatment Under Study on pages 5 through 6). 适当的临床试验的初步治疗（ 见根据治疗的研究上5至6页） 。
Treatment for Patients With Relapsed or Refractory PTCL 治疗复发或难治性PTCL的患者
Pralatrexate (Folotyn Pralatrexate（Folotyn
® ®
), a metabolic inhibitor that has been shown to reduce tumor size, is FDA ），代谢抑制剂，已被证明，以减少肿瘤的大小，是 FDA
approved to treat relapsed or refractory PTCL. 批准用于治疗复发或难治性PTCL的。 There are no other FDA-approved drugs specifically 有没有其他FDA批准的药物专门
indicated for PTCL, nor has a common standard of care been identified for patients with relapsed or 表示PTCL的，也有照顾的共同标准，被确定患者复发或
refractory PTCL. 难治性PTCL的。 Patients with relapsed or refractory disease should consult with the members of their 复发或难治性疾病的患者应咨询其成员
medical team for information about participating in an appropriate clinical trial. 医疗团队参加适当的临床试验的信息 。

Page 5 第5页
The Leukemia & Lymphoma Society • 1311 Mamaroneck Avenue • White Plains, NY 10605 白血病和淋巴瘤协会 •1311马马罗内克大道•白色平原，纽约10605
Information Specialist 800.955.4572 • www.LLS.org 信息专家800.955.4572•www.LLS.org
Peripheral T-Cell Lymphoma Facts/5 外周T细胞淋巴瘤的事实/ 5
Some common chemotherapy-based regimens used to treat patients with relapsed or refractory disease are 一些常见的化疗方案用于治疗复发或难治性疾病的患者
• ICE (ifosfamide, carboplatin, etoposide) •ICE（异环磷酰胺，卡铂，依托泊苷）
• DHAP (high-dose cytarabine [ara-C], dexamethasone, cisplatin [Platinol •DHAP（大剂量阿糖胞苷的Ara - C]，地塞米松，顺铂[Platinol
® ®
-AQ]) - AQ]）
• ESHAP (etoposide, methylprednisolone, cytarabine [ara-C], ci