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Home , Ann Arbor staging, Belinostat, Mantle cell lymphoma

Peripheral T-Cell Facts No. 25 in a series providing the latest information for patients, caregivers and healthcare professionals

www.LLS.org • Information Specialist: 800.955.4572

Highlights Introduction Peripheral T-cell (PTCLs) are uncommon and l Peripheral T-cell lymphomas (PTCLs) comprise a aggressive types of non- (NHL) that diverse group of uncommon and aggressive diseases develop in mature white blood cells called “T cells” and in which the patient’s T cells become cancerous. T-cell “natural killer (NK) cells.” lymphomas account for between 10 percent and 15 percent of all non-Hodgkin lymphomas (NHLs). NHL is the name for many different types of that l The World Health Organization (WHO) divides start in cells called “lymphocytes,” a type of white blood PTCLs into three categories (nodal, extranodal cell that helps the body fight infection. There are three and leukemic) and classified subtypes within these types of lymphocytes: B lymphocytes (B cells), categories of PTCLs. Getting an accurate diagnosis T lymphocytes (T cells) and natural killer cells (NK cells). and knowing your PTCL subtype is important. NHL may arise in B cells or T cells. B-cell lymphomas are more common than T-cell lymphomas. NHLs may be l PTCLs are rare in the United States and are more indolent (slow growing) or aggressive (fast growing). For common in Asia, Africa and the Caribbean, possibly more information about NHL, please see the free due to exposure to specific viruses, such as the & Lymphoma Society (LLS) booklets Non-Hodgkin Epstein-Barr virus (EBV) and the human T-cell Lymphoma and The Lymphoma Guide – Information for leukemia virus-1 (HTLV-1). Patients and Caregivers. l PTCLs generally affect people older than 60 years, although they can occur anytime during adulthood. This publication provides descriptions of the various subtypes of PTCL. It also includes specific information on l Although the signs and symptoms of PTCLs vary the diagnosis, stages and treatment of PTCLs; new drugs according to the subtype, some common signs and being studied in clinical trials; and support resources. symptoms of the diseases include fatigue, a painless swelling in the neck, armpit or groin (due to an enlarged ), , rash and . About Peripheral T-Cell Lymphoma l New therapies are showing some effectiveness in Between 10 percent and 15 percent of all patients with treating patients who have certain subtypes of PTCL, NHL have a T-cell lymphoma subtype. PTCLs generally and other potential therapies are being studied in affect people aged 60 years and older and are diagnosed clinical trials. However, standards of care have not slightly more often in men than in women. However, been established for newly diagnosed PTCL patients younger adults and children are also diagnosed with PTCLs. or for patients who have disease that has relapsed PTCL is an uncommon disease in the United States. Some (recurred) or is refractory (resistant to treatment). forms of PTCL are more common in Asia, Africa and the Caribbean, possibly as a result of exposure to specific l More research and clinical trials focusing specifically on the various subtypes of PTCL are needed to define viruses, such as the Epstein-Barr virus (EBV) and the the best management of patients who have these human T-cell leukemia virus-1 (HTLV-1). diseases. The World Health Organization (WHO) classification system recognizes subtypes of PTCL and has grouped the diseases into three categories: nodal, extranodal and leukemic. WHO has also divided T-cell lymphomas into two groups: aggressive (fast growing) and indolent (slow growing). PTCLs are a varied group of diseases that differ from B-cell This publication was supported in part by a grant from lymphomas. Because PTCLs are less common than B-cell

FS25 Peripheral T-Cell Lymphoma Facts I page 1 Revised July 2014 Peripheral T-Cell Lymphoma Facts lymphomas, they are not as well understood. Techniques so that the hematopathologist (a doctor who specializes in to distinguish and study the various subtypes of PTCL interpreting and diagnosing the physical changes caused have only recently been developed. As a result, standards of by diseases of the blood and marrow) has enough tissue to care for how best to treat PTCLs have not been established make a firm diagnosis. Lymph node tissue can often for newly diagnosed patients or for patients whose be removed after the administration of a local anesthetic. disease has relapsed (recurred) or is refractory (resistant The cells in many subtypes of PTCLs look alike; therefore, to treatment). In general, treatment outcomes have been making an accurate diagnosis may require the use of poor with conventional regimens. However, additional diagnostic tests, including blood tests, CT a greater understanding of PTCLs and new genetic and (computerized axial tomography), PET (positron emission molecular testing techniques have led to the development tomography) scans, MRI (magnetic resonance imaging), of new targeted drugs (see Treatment Under Study on and bone marrow biopsy. page 4). Other therapies are being explored and tested in research laboratories and in human clinical trials designed An accurate diagnosis is the start for planning the treatment specifically for T-cell lymphomas (see Peripheral T-Cell approach. An experienced hematopathologist is needed Lymphoma Subtypes on pages 5 and 6). to analyze the biopsy slides. A second opinion by another hematopathologist may be necessary if there is any doubt Because PTCLs are so uncommon, it is best to seek about the diagnosis. treatment at a medical center specializing in the diagnosis and treatment of NHL (see the free LLS fact sheet Choosing a Blood Cancer Specialist or Treatment Center for more Treatment Planning information). Every patient’s medical situation is different and should be Another group of T-cell lymphomas is called “cutaneous evaluated individually by an oncologist who specializes in T-cell lymphomas (CTCLs),” or skin lymphomas. CTCLs treating NHL. It is important for you and members of your consist of a number of different diseases with various signs team to discuss all treatment options, including and symptoms, treatment approaches and outcomes. While treatments being studied in clinical trials. there may be skin involvement with some PTCLs, CTCLs originate in the skin. CTCLs are primarily slow growing. Staging This group of diseases is described in detail in the free LLS Knowing the stage of your disease helps members of your fact sheet Cutaneous T-Cell Lymphoma Facts. health care team determine the most effective course of treatment for you. The Ann Arbor Staging System is the most Signs and Symptoms common system used for classifying all subtypes of NHL. The first signs of PTCL vary depending on the disease The system is divided into four stages and is based on where subtype. Because lymph nodes in several different areas of the disease is located in the body: the body are frequently involved, the most common sign of PTCL is an enlarged, painless lymph node in the neck, l Stage I—The cancer is in a single lymph node or lymph armpit or groin. Enlarged lymph nodes can also appear near node region or, the cancer is in an organ or site other than a the ears or elbows. These lymphomas also affect various lymph node (extranodal) but has not spread to other organs organs in the body, including the bone marrow, liver, or lymph nodes. , stomach and skin. Other symptoms may include l Stage II—The cancer is in two or more lymph node regions l Night sweats on the same side of the diaphragm. l l Stage III—The cancer is in lymph node regions on both l Weight loss sides of the diaphragm, with or without partial involve- ment of an extranodal organ or site above or below the l Rash. diaphragm.

Diagnosis l Stage IV—The cancer is widespread, including multiple Most PTCLs are diagnosed by taking a small sample (a involvements in one or more extranodal sites, such as the “biopsy”) of an enlarged lymph node and then examining bone marrow. the cells under a microscope. Generally, either the lymph node, or a part of the lymph node, is surgically removed

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Treatment for Newly Diagnosed The number of IPI ‘risk factors’ a person has defines the IPI risk group to help predict the risk of relapse. Each point Patients represents some increased risk for disease recurrence. The Currently, newly diagnosed PTCL patients are usually total number of points identifies the following risk groups: treated with -based chemotherapy regimens. low risk (0-1 points); low-intermediate risk (2 points); Most subtypes of PTCL are treated as follows high-intermediate risk (3 points); high risk (4-5 points). For l CHOP (, hydroxydoxorubicin patients younger than 60 years, the risk categories are slightly [], Oncovin® [], ) different; low risk (0 points); low-intermediate risk (1 point); high-intermediate risk (2 points); high risk (3 points). l CHOEP (cyclophosphamide, hydroxydoxorubicin [doxorubicin], Oncovin® [vincristine], , The Prognostic Index for PTCL (PIT). PIT is a prognostic prednisone) index used mainly for peripheral T-cell lymphoma, not l EPOCH (etoposide, prednisone, Oncovin® [vincristine], otherwise specified (PTCL-NOS). PIT separates PTCL- cyclophosphamide, hydroxydoxorubicin [doxorubicin]) NOS patients into more specific prognostic groups than the IPI. PIT is based on four risk factors: age, performance l Hyper-CVAD (cyclophosphamide, Oncovin® status, serum (LDH) and bone [vincristine], Adriamycin® [doxorubicin], dexamethasone); marrow involvement. By using these risk factors instead “hyper” refers to “hyperfractionation of the dose,” of the risk factors associated with the IPI, PIT has a better meaning that the chemotherapy is given in small, frequent predictive capacity for PTCL-NOS. doses to minimize side effects. Patients may want to discuss risk factors with their doctor l Clinical trials with new combinations of in order to understand treatment options, including (preferred). participation in clinical trials. Unfortunately, cure rates for PTCL remain low with the exception of the cure rates for Treatment for Patients With Relapsed l ALK-positive anaplastic large cell lymphoma (see or Refractory PTCL Peripheral T-Cell Lymphoma Subtypes on pages 5 and 6) A common standard of care has not been identified for l Localized extranodal NK/T-cell lymphoma, for patients with relapsed or refractory PTCL. Patients with which localized radiotherapy and anthracycline-based relapsed or refractory disease should consult with the chemotherapy are usually recommended. members of their medical team for information about Patients with PTCL should consult with the members of participating in an appropriate clinical trial. their medical team about the availability of appropriate Drugs that are currently FDA approved to treat relapsed or clinical trials for initial treatment (see Treatment Under refractory PTCL include Study on page 4). l Belinostat (BeleodaqTM), a (HDAC) The International Prognostic Index (IPI). The IPI is inhibitor, given intravenously (IV) a scoring system that uses known risk factors to predict l  (Folotyn®), a metabolic inhibitor that has overall survival and guide treatment decisions. This been shown to reduce tumor size and is given by IV information helps doctors to determine appropriate care for l  (Istodax®), a histone deacetylase (HDAC) patients who have been treated for aggressive lymphomas inhibitor, given by IV. Istodax is approved for treatment and predict risk of relapse. in patients who have received at least one prior therapy. One point is assigned for each of the following risk factors Some common chemotherapy-based regimens used to treat l Age greater than 60 years patients with relapsed or refractory disease are l Stage III or IV disease l ICE (, , etoposide) l More than one lymph node involved l DHAP (high-dose [ara-C], dexamethasone, l Elevated serum lactate dehydrogenase (LDH) [Platinol®-AQ]) l Performance status, which uses a scale to evaluate a l ESHAP (etoposide, methylprednisolone, cytarabine person’s ability to perform daily tasks of living without [ara-C], cisplatin [Platinol®-AQ]) help. l GND (, navelbine, dexamethasone) or other gemcitabine containing regimens.

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Stem Cell Transplantation “epigenetic” changes (changes that affect certain cell processes without changing the genetic makeup of the Consolidation therapy, which consists of high-dose cell). Therapies include (Zolinza®), already chemotherapy followed by a stem cell transplant, is often approved for the treatment of patients who have recommended for patients in first-time remission (no cutaneous T-cell lymphoma, belinostat (Beleodaq™) and evidence of disease detected with standard tests). The (LBH-589). exceptions are specific PTCL patients who are considered to be at low risk for relapse and patients with ALK-positive l Proteasome inhibitors, which block the action of anaplastic large cell lymphoma, who usually have a good proteasomes. A proteasome is a large cell protein that prognosis. helps destroy other cell proteins when they are no longer needed. (Velcade®), an agent that is approved There are two types of stem cell transplants: autologous, in to treat patients with myeloma or mantle cell lymphoma, which patients receive their own stem cells, and allogeneic, is currently being evaluated in clinical trials both as in which patients receive stem cells from a matched donor. a single agent and in combination with conventional Both types of transplants are used to treat PTCLs. The use chemotherapy. of autologous versus allogeneic transplant is being studied l Immunomodulatory drugs, a novel class of small-molecule to know what the best option is for individual patients. anticancer and anti-inflammatory drugs with broad Because the high-dose chemotherapy regimens used with biologic activities. (Revlimid®), stem cell transplantation can cause serious side effects or FDA approved to treat patients with myeloma or complications, including bone marrow suppression and myelodysplastic syndromes, is currently being studied to infections, stem cell transplants are not a treatment option treat PTCL patients. for everyone with PTCL. To determine whether you are a l Monoclonal antibodies, types of protein that can bind good candidate for a transplant, members of your medical to tumor cells. Therapies being investigated for PTCL team will consider your include alemtuzumab (Campath®), which is already approved in the treatment of chronic lymphocytic l Medical history leukemia. l General health l Nucleoside analogs, which activate pathways that prevent l Cancer stage progression and repair DNA. Two examples l Response to previous treatment of nucleoside analogs being studied in the treatment l Age. of PTCL are gemcitabine (Gemzar®), approved in the treatment of several solid tumor including There is some evidence that the use of reduced-intensity ovarian and breast cancer, and (Arranon®). conditioning (RIC) prior to a stem cell transplant may be Arranon® is approved by the FDA for the treatment of a good alternative to high-dose chemotherapy for some relapsed or refractory PTCL subtype precursor T-cell PTCL patients who may be at increased risk for developing acute lymphoblastic leukemia and precursor T-cell acute treatment-related toxicities. However, larger studies lymphoblastic lymphoma in adults and children. using RIC in PTCL patients are needed to determine the effectiveness of the treatment. See the free LLS booklet Treatment Outcomes Blood and Marrow Stem Cell Transplantation for more information. Peripheral T-cell lymphomas comprise a group of rare and diverse diseases that are difficult to cure. As a result, standard of care for how best to treat PTCLs in newly diagnosed Treatment Under Study patients or in patients whose disease has relapsed (recurred) Until recently, treatment approaches for patients with or become refractory (does not respond to treatment) has not any type of PTCL were similar to treatment regimens been established. However, many new agents currently being developed for patients with B-cell lymphomas. However, tested in clinical trials (see Treatment Under Study on this these treatments have proven to be largely ineffective for page) are showing encouraging responses in the treatment of PTCL patients. New classes of therapies that target specific PTCLs. molecular pathways of T-cell lymphomas are being studied Because PTCLs are so rare and may be difficult to treat, it is in clinical trials. Some of the classes of novel therapies and best to seek treatment at a medical center specializing in the drugs under investigation include diagnosis and treatment of NHL. Your medical team will l Histone deacetylase (HDAC) inhibitors, which target then discuss what your treatment options are and whether

FS25 Peripheral T-Cell Lymphoma Facts I page 4 Peripheral T-Cell Lymphoma Facts a clinical trial is right for you. If you need help locating a Specialist by calling (800) 955-4572 or by going to the LLS medical center near you or if you need assistance finding website at www.LLS.org/informationspecialists. an appropriate clinical trial, contact an LLS Information

Peripheral T-Cell Lymphoma good response to the chemotherapy combination CHOP (cyclophosphamide, hydroxydoxorubicin Subtypes [doxorubicin], Oncovin® [vincristine], prednisone) Peripheral T-Cell Lymphoma, Not Otherwise and other similar chemotherapy combinations Specified (NOS)—Peripheral T-cell lymphoma, and can achieve long-term remission or cure. NOS is the most common type of PTCL and Brentuximab vedotin, (Adcetris®) also known comprises a group of mixed T-cell diseases that do as SGN-35, is FDA approved for the treatment not fit into any of the other subtypes of PTCL. of patients with systemic anaplastic large cell Most patients with PTCL-NOS will have nodal lymphoma (ALCL) after failure of at least one involvement, but extranodal sites, such as the liver, prior multi-agent . bone marrow, gastrointestinal tract and skin, may Brentuximab vedotin is administered by injection. also be involved. This group of PTCLs is considered ALK-negative patients usually relapse and may need aggressive and in the past has usually been treated more aggressive treatment, including high-dose with standard CHOP (cyclophosphamide, chemotherapy and a stem cell transplant. hydroxydoxorubicin [doxorubicin], Oncovin® Anaplastic Large Cell Lymphoma (Primary [vincristine], prednisone) chemotherapy at initial Cutaneous)—Primary cutaneous ALCL is thought diagnosis. However, since the chemotherapy to be a more indolent (slow-growing) lymphoma and combination CHOP has not produced very good typically affects the skin. This lymphoma is usually outcomes, doctors are currently evaluating other ALK negative, although the prognosis is fairly good. chemotherapy combinations for initial therapy. Other drugs that may be effective in treating Angioimmunoblastic T-Cell Lymphoma— patients who have PTCL-NOS include gemcitabine Angioimmunoblastic T-cell lymphoma (AITL) (Gemzar®) and bortezomib (Velcade®), which are accounts for between 1 percent and 2 percent showing response rates of about 60 percent and 30 of all cases of NHL and typically follows an percent, respectively. A number of small studies aggressive course, although spontaneous disease have demonstrated disease-free survival rates of regression sometimes occurs. AITL usually occurs between 35 percent and 45 percent in some patients in the lymph nodes and may affect the spleen or who received high-dose chemotherapy followed liver. Some symptoms may include fever, weight by autologous stem cell transplantation. However, loss and rashes. The chemotherapy combination larger studies need to be conducted to more CHOP (cyclophosphamide, hydroxydoxorubicin accurately assess the long-term effectiveness of this [doxorubicin], Oncovin® [vincristine], prednisone) type of treatment. Clinical trials evaluating newer has also been commonly used for this subtype of combinations of therapies are ongoing. lymphoma, but the results do not produce many long-term disease-free survivors. For this subtype Anaplastic Large Cell Lymphoma—This rare of lymphoma, new chemotherapy combinations T-cell lymphoma constitutes about 3 percent of either with or without stem cell transplantation are all cases of lymphomas in adults and between 10 being evaluated. For patients with relapsed disease, and 30 percent of all lymphomas in children. It therapies such as immunosuppressive agents or usually affects nodal sites, although extranodal targeted agents are being evaluated in clinical trials. sites can also be involved. Anaplastic large cell lymphoma (ALCL) is divided into two major Nasal, Natural Killer (NK)/T-cell Lymphoma— subtypes based on the presence or absence of a Extranodal nasal, NK/T-cell lymphoma typically protein called “anaplastic lymphoma kinase (ALK).” affects the nasal area and the paranasal sinus Patients with ALK-positive disease usually have a areas behind the nose and cheeks, although the

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lymphoma can also occur at extranodal sites such Hepatosplenic Lymphoma—This type of T-cell as the skin, gastrointestinal tract and testes. The lymphoma is an extremely rare and aggressive disease is rare in the United States, accounting disease that starts in the liver or spleen and usually for less than 1.5 percent of all NHLs. Extranodal, affects young adults in their 20s and 30s. Treatment nasal NK/T-cell lymphoma is more commonly for patients with hepatosplenic T-cell lymphoma found in Asia and Latin America and is associated includes anthracycline-based chemotherapy and, in with the Epstein-Barr virus (EBV). Radiation and some cases, stem cell transplantation. chemotherapy treatments are usually recommended Subcutaneous Panniculitis-Like Lymphoma— for localized nasal NK/T-cell disease. Subcutaneous panniculitis-like T-cell lymphoma Adult T-Cell Acute Lymphoblastic Lymphoma (SPTCL) is the rarest and least well-defined of the or Leukemia—Adult T-cell acute lymphoblastic T-cell lymphomas. This lymphoma occurs primarily lymphoma or leukemia (ATLL), which is more in the subcutaneous fat tissue, where it causes commonly found in Japan and the Caribbean than nodules to form. Symptoms include fever, chills, in the United States, is associated with the human weight loss and oral mucosal ulcers. SPTCL may be T-cell leukemia virus-1 (HTLV-1). The HTLV-1 either rapidly aggressive or indolent (slow growing). virus is transmitted through sexual intercourse, Treatment includes combination anthracycline-based childbirth, blood transfusions, shared needles and chemotherapy or localized radiation. breast milk. Although people who have ATLL Precursor T-Cell Acute Lymphoblastic Lymphoma may initially respond to chemotherapy, the or Leukemia—This subtype of PTCL may be long-term prognosis is poor. Clinical trials are diagnosed as leukemia or lymphoma or both. This under way to investigate adding agents such as cancer is found in both children and adults and is and bortezomib (Velcade®) as well as most commonly diagnosed in adolescent and adult treatments ranging from stem cell transplantation to males. Treatment for newly diagnosed patients with conventional chemotherapy regimens. precursor T-cell acute lymphoblastic lymphoma or Enteropathy-Associated Lymphoma—This T-cell leukemia is aggressive chemotherapy and radiation. lymphoma is associated with celiac disease, a chronic Nelarabine (Arranon®) is approved for the treatment intestinal disorder caused by a hypersensitivity to of relapsed or refractory precursor T-cell acute gluten proteins found in wheat, rye and barley. lymphoblastic lymphoma or leukemia in adults and Symptoms usually include stomach pain, weight children. loss, gastrointestinal bleeding or bowel perforation. Blastic NK-Cell Lymphoma—This T-cell Treatment for patients with enteropathy-associated lymphoma is very rare, very fast growing and T-cell lymphoma includes an anthracycline-based difficult to treat. Patients with blastic NK-cell chemotherapy regimen, nutritional supplements lymphoma should talk to their medical team about and, if appropriate, a gluten-free diet. participating in promising clinical trials.

Acknowledgement We’re Here to Help LLS gratefully acknowledges LLS is the world’s largest voluntary health organization Julie Vose, MD dedicated to funding blood , education and Professor of Medicine, Division of and Oncology patient services. LLS has chapters throughout the United The University of Nebraska Medical Center States and in Canada. To find the chapter nearest to you, Omaha, Nebraska visit our website at www.LLS.org or contact for her review of Peripheral T-Cell Lymphoma Facts and her The Leukemia & Lymphoma Society important contributions to the material presented in this 1311 Mamaroneck Avenue publication. Suite 310 White Plains, NY 10605 Contact an Information Specialist at (800) 955-4572 Email: [email protected]

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LLS offers free information and services for patients and l Call: (800) 955-4572 families touched by blood cancers. The following lists l Visit: www.LLS.org/chapterfind. various resources available to you. Use this information to learn more, to ask questions, and to make the most of your Clinical Trials (Research Studies). New treatments for healthcare team. patients with PTCL are under way. Many are part of clinical trials. Patients can learn about clinical trials and how to Consult with an Information Specialist. Information access them. Specialists are master’s level oncology social workers, nurses and health educators. They can answer general questions For more information, please about diagnosis and treatment options, offer guidance and l Call: (800) 955-4572 to speak with our LLS Information support and assist with clinical-trials searches. Language Specialists who can help conduct clinical trial searches services are available. l Visit: www.LLS.org/clinicaltrials. For more information, please Advocacy. The LLS Office of Public Policy (OPP) enlists l Call: (800) 955-4572 (M-F, 9 a.m. to 9 p.m. EST) volunteers to advocate for policies and laws to speed new treatments and improve access to quality medical care. l Email: [email protected] l Live chat: www.LLS.org For more information, please l Visit: www.LLS.org/informationspecialists. l Call: (800) 955-4572 Free Materials. LLS offers free education and support l Visit: www.LLS.org/advocacy. publications that can either be read online or downloaded. Free print versions can be ordered. Other Resources For more information, please visit www.LLS.org/publications. The National Cancer Institute (800) 422-6237 Telephone/Web Education Programs. LLS offers free www.cancer.gov telephone/Web education programs for patients, caregivers The National Cancer Institute is part of the National and healthcare professionals. Institutes of Health and is a national resource center for For more information, please visit www.LLS.org/programs. information and education about all forms of cancer, including peripheral T-cell lymphomas (PTCLs). The Co-Pay Assistance Program. LLS offers insurance premium NCI also provides a clinical-trials search feature, the and co-pay assistance for certain eligible patients. PDQ®Cancer Clinical Trials Registry, at the website For more information, please www.cancer.gov/clinicaltrials/search-form-help, where PTCL patients can look for clinical trials for their specific l Call: (877) 557-2672 subtype. l Visit: www.LLS.org/copay. FocusonPTCL.org Online Blood Cancer Discussion Boards and Chats. FocusonPTCL.org (focusonptcl.org) offers comprehensive Online discussion boards and moderated online chats can information on PTCLs, including treatment options. help cancer patients to reach out, share information and provide support. References For more information, please visit www.LLS.org/getinfo. Chen AI, Advani RH. Beyond the guidelines in the LLS Chapters. LLS offers support and services in the treatment of peripheral T-cell lymphoma: new drug United States and Canada including the Patti Robinson development. Journal of the National Comprehensive Cancer Kaufmann First Connection Program (a peer-to-peer support Network. 2008;6(4):428-435. program), in-person support groups, and other great Foss F. Evolving therapy of peripheral T-cell lymphoma. resources. Therapeutic Advances in Hematology. 2011;2(3):161-173. For more information about these programs or to contact Foss FM, Zinzani PL, Vose JM, et al. Peripheral T-cell your chapter, please lymphoma. Blood. 2011;117(25):6756-6767.

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Gallamini A, Stelitano C, Calvi R et al. Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicenter clinical study. Blood. 2004;103(7):2474-2479. Liess DB, Templer JW. NK-cell lymphomas of the head and neck. Updated October 15, 2012. www.emedicine.medscape.com. Accessed April 2, 2014. Savage KJ. Aggressive peripheral T-cell lymphomas (specified and unspecified types). Hematology/the Education Program of the American Society of Hematology. 2005; 267-277. Schmitz N, Trumper L, Ziepert M, et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German high-grade non-Hodgkin lymphoma study group. Blood. 2010;116(18):3418-3425. Skarbnik AP, Burki M and Pro B. Peripheral T-cell lymphomas: a review of current approaches and hopes for the future. Frontiers in Oncology. 2013;3(138):1-9. Vose JM. Peripheral T-cell Non-Hodgkin’s lymphoma. Hematology/oncology Clinics of North America. 2008;22(5):997-1005.

This publication is designed to provide accurate and authoritative information in regard to the subject matter covered. It is distributed as a public service by The Leukemia & Lymphoma Society (LLS), with the understanding that The Leukemia & Lymphoma Society is not engaged in rendering medical or other professional services.

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