FS25 Peripheral T-Cell Lymphoma Facts I page 1
Revised July 2014
Introduction
Peripheral T-cell lymphomas (PTCLs) are uncommon and
aggressive types of non-Hodgkin lymphoma (NHL) that
develop in mature white blood cells called “T cells” and
“natural killer (NK) cells.”
NHL is the name for many dierent types of cancer that
start in cells called “lymphocytes,” a type of white blood
cell that helps the body ght infection. ere are three
types of lymphocytes: B lymphocytes (B cells),
T lymphocytes (T cells) and natural killer cells (NK cells).
NHL may arise in B cells or T cells. B-cell lymphomas
are more common than T-cell lymphomas. NHLs may be
indolent (slow growing) or aggressive (fast growing). For
more information about NHL, please see the free Leukemia
& Lymphoma Society (LLS) booklets Non-Hodgkin
Lymphoma and e Lymphoma Guide – Information for
Patients and Caregivers.
is publication provides descriptions of the various
subtypes of PTCL. It also includes specic information on
the diagnosis, stages and treatment of PTCLs; new drugs
being studied in clinical trials; and support resources.
About Peripheral T-Cell Lymphoma
Between 10 percent and 15 percent of all patients with
NHL have a T-cell lymphoma subtype. PTCLs generally
aect people aged 60 years and older and are diagnosed
slightly more often in men than in women. However,
younger adults and children are also diagnosed with PTCLs.
PTCL is an uncommon disease in the United States. Some
forms of PTCL are more common in Asia, Africa and
the Caribbean, possibly as a result of exposure to specic
viruses, such as the Epstein-Barr virus (EBV) and the
human T-cell leukemia virus-1 (HTLV-1).
e World Health Organization (WHO) classication
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 dier from B-cell
lymphomas. Because PTCLs are less common than B-cell
Peripheral
T-Cell Lymphoma Facts
www.LLS.org • Information Specialist: 800.955.4572
Highlights
l Peripheral T-cell lymphomas (PTCLs) comprise a
diverse group of uncommon and aggressive diseases
in which the patient’s T cells become cancerous. T-cell
lymphomas account for between 10 percent and 15
percent of all non-Hodgkin lymphomas (NHLs).
l e World Health Organization (WHO) divides
PTCLs into three categories (nodal, extranodal
and leukemic) and classied subtypes within these
categories of PTCLs. Getting an accurate diagnosis
and knowing your PTCL subtype is important.
l PTCLs are rare in the United States and are more
common in Asia, Africa and the Caribbean, possibly
due to exposure to specic viruses, such as the
Epstein-Barr virus (EBV) and the human T-cell
leukemia virus-1 (HTLV-1).
l PTCLs generally aect people older than 60 years,
although they can occur anytime during adulthood.
l Although the signs and symptoms of PTCLs vary
according to the subtype, some common signs and
symptoms of the diseases include fatigue, a painless
swelling in the neck, armpit or groin (due to an enlarged
lymph node), night sweats, rash and weight loss.
l New therapies are showing some eectiveness in
treating patients who have certain subtypes of PTCL,
and other potential therapies are being studied in
clinical trials. However, standards of care have not
been established for newly diagnosed PTCL patients
or for patients who have disease that has relapsed
(recurred) or is refractory (resistant to treatment).
l More research and clinical trials focusing specically
on the various subtypes of PTCL are needed to dene
the best management of patients who have these
diseases.
is publication was supported in part by a grant from
No. 25 in a series providing the latest information for
patients, caregivers and healthcare professionals
FS25 Peripheral T-Cell Lymphoma Facts I page 2
Peripheral T-Cell Lymphoma Facts
lymphomas, they are not as well understood. Techniques
to distinguish and study the various subtypes of PTCL
have only recently been developed. As a result, standards of
care for how best to treat PTCLs have not been established
for newly diagnosed patients or for patients whose
disease has relapsed (recurred) or is refractory (resistant
to treatment). In general, treatment outcomes have been
poor with conventional chemotherapy regimens. However,
a greater understanding of PTCLs and new genetic and
molecular testing techniques have led to the development
of new targeted drugs (see Treatment Under Study on
page 4). Other therapies are being explored and tested in
research laboratories and in human clinical trials designed
specically for T-cell lymphomas (see Peripheral T-Cell
Lymphoma Subtypes on pages 5 and 6).
Because PTCLs are so uncommon, it is best to seek
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
information).
Another group of T-cell lymphomas is called “cutaneous
T-cell lymphomas (CTCLs),” or skin lymphomas. CTCLs
consist of a number of dierent diseases with various signs
and symptoms, treatment approaches and outcomes. While
there may be skin involvement with some PTCLs, CTCLs
originate in the skin. CTCLs are primarily slow growing.
is group of diseases is described in detail in the free LLS
fact sheet Cutaneous T-Cell Lymphoma Facts.
Signs and Symptoms
e rst signs of PTCL vary depending on the disease
subtype. Because lymph nodes in several dierent areas of
the body are frequently involved, the most common sign
of PTCL is an enlarged, painless lymph node in the neck,
armpit or groin. Enlarged lymph nodes can also appear near
the ears or elbows. ese lymphomas also aect various
organs in the body, including the bone marrow, liver,
spleen, stomach and skin. Other symptoms may include
l Night sweats
l Fever
l Weight loss
l Rash.
Diagnosis
Most PTCLs are diagnosed by taking a small sample (a
“biopsy”) of an enlarged lymph node and then examining
the cells under a microscope. Generally, either the lymph
node, or a part of the lymph node, is surgically removed
so that the hematopathologist (a doctor who specializes in
interpreting and diagnosing the physical changes caused
by diseases of the blood and marrow) has enough tissue to
make a rm diagnosis. Lymph node biopsy tissue can often
be removed after the administration of a local anesthetic.
e cells in many subtypes of PTCLs look alike; therefore,
making an accurate diagnosis may require the use of
additional diagnostic tests, including blood tests, CT
(computerized axial tomography), PET (positron emission
tomography) scans, MRI (magnetic resonance imaging),
and bone marrow biopsy.
An accurate diagnosis is the start for planning the treatment
approach. An experienced hematopathologist is needed
to analyze the biopsy slides. A second opinion by another
hematopathologist may be necessary if there is any doubt
about the diagnosis.
Treatment Planning
Every patient’s medical situation is dierent and should be
evaluated individually by an oncologist who specializes in
treating NHL. It is important for you and members of your
oncology team to discuss all treatment options, including
treatments being studied in clinical trials.
Staging
Knowing the stage of your disease helps members of your
health care team determine the most eective course of
treatment for you. e Ann Arbor Staging System is the most
common system used for classifying all subtypes of NHL.
e system is divided into four stages and is based on where
the disease is located in the body:
l Stage I—e cancer is in a single lymph node or lymph
node region or, the cancer is in an organ or site other than a
lymph node (extranodal) but has not spread to other organs
or lymph nodes.
l Stage II—e cancer is in two or more lymph node regions
on the same side of the diaphragm.
l Stage III—e cancer is in lymph node regions on both
sides of the diaphragm, with or without partial involve-
ment of an extranodal organ or site above or below the
diaphragm.
l Stage IV—e cancer is widespread, including multiple
involvements in one or more extranodal sites, such as the
bone marrow.
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Peripheral T-Cell Lymphoma Facts
Treatment for Newly Diagnosed
Patients
Currently, newly diagnosed PTCL patients are usually
treated with anthracycline-based chemotherapy regimens.
Most subtypes of PTCL are treated as follows
l CHOP (cyclophosphamide, hydroxydoxorubicin
[doxorubicin], Oncovin® [vincristine], prednisone)
l CHOEP (cyclophosphamide, hydroxydoxorubicin
[doxorubicin], Oncovin® [vincristine], etoposide,
prednisone)
l EPOCH (etoposide, prednisone, Oncovin® [vincristine],
cyclophosphamide, hydroxydoxorubicin [doxorubicin])
l Hyper-CVAD (cyclophosphamide, Oncovin®
[vincristine], Adriamycin® [doxorubicin], dexamethasone);
“hyper” refers to “hyperfractionation of the dose,”
meaning that the chemotherapy is given in small, frequent
doses to minimize side eects.
l Clinical trials with new combinations of chemotherapies
(preferred).
Unfortunately, cure rates for PTCL remain low with the
exception of the cure rates for
l ALK-positive anaplastic large cell lymphoma (see
Peripheral T-Cell Lymphoma Subtypes on pages 5 and 6)
l Localized extranodal NK/T-cell lymphoma, for
which localized radiotherapy and anthracycline-based
chemotherapy are usually recommended.
Patients with PTCL should consult with the members of
their medical team about the availability of appropriate
clinical trials for initial treatment (see Treatment Under
Study on page 4).
e International Prognostic Index (IPI). e IPI is
a scoring system that uses known risk factors to predict
overall survival and guide treatment decisions. is
information helps doctors to determine appropriate care for
patients who have been treated for aggressive lymphomas
and predict risk of relapse.
One point is assigned for each of the following risk factors
l Age greater than 60 years
l Stage III or IV disease
l More than one lymph node involved
l Elevated serum lactate dehydrogenase (LDH)
l Performance status, which uses a scale to evaluate a
person’s ability to perform daily tasks of living without
help.
e number of IPI ‘risk factors’ a person has denes the IPI
risk group to help predict the risk of relapse. Each point
represents some increased risk for disease recurrence. e
total number of points identies the following risk groups:
low risk (0-1 points); low-intermediate risk (2 points);
high-intermediate risk (3 points); high risk (4-5 points). For
patients younger than 60 years, the risk categories are slightly
dierent; low risk (0 points); low-intermediate risk (1 point);
high-intermediate risk (2 points); high risk (3 points).
e Prognostic Index for PTCL (PIT). PIT is a prognostic
index used mainly for peripheral T-cell lymphoma, not
otherwise specied (PTCL-NOS). PIT separates PTCL-
NOS patients into more specic prognostic groups than
the IPI. PIT is based on four risk factors: age, performance
status, serum lactate dehydrogenase (LDH) and bone
marrow involvement. By using these risk factors instead
of the risk factors associated with the IPI, PIT has a better
predictive capacity for PTCL-NOS.
Patients may want to discuss risk factors with their doctor
in order to understand treatment options, including
participation in clinical trials.
Treatment for Patients With Relapsed
or Refractory PTCL
A common standard of care has not been identied for
patients with relapsed or refractory PTCL. Patients with
relapsed or refractory disease should consult with the
members of their medical team for information about
participating in an appropriate clinical trial.
Drugs that are currently FDA approved to treat relapsed or
refractory PTCL include
l Belinostat (Beleodaq
TM
), a histone deacetylase (HDAC)
inhibitor, given intravenously (IV)
l Pralatrexate (Folotyn®), a metabolic inhibitor that has
been shown to reduce tumor size and is given by IV
l Romidepsin (Istodax®), a histone deacetylase (HDAC)
inhibitor, given by IV. Istodax is approved for treatment
in patients who have received at least one prior therapy.
Some common chemotherapy-based regimens used to treat
patients with relapsed or refractory disease are
l ICE (ifosfamide, carboplatin, etoposide)
l DHAP (high-dose cytarabine [ara-C], dexamethasone,
cisplatin [Platinol®-AQ])
l ESHAP (etoposide, methylprednisolone, cytarabine
[ara-C], cisplatin [Platinol®-AQ])
l GND (gemcitabine, navelbine, dexamethasone) or other
gemcitabine containing regimens.
FS25 Peripheral T-Cell Lymphoma Facts I page 4
Peripheral T-Cell Lymphoma Facts
Stem Cell Transplantation
Consolidation therapy, which consists of high-dose
chemotherapy followed by a stem cell transplant, is often
recommended for patients in rst-time remission (no
evidence of disease detected with standard tests). e
exceptions are specic PTCL patients who are considered
to be at low risk for relapse and patients with ALK-positive
anaplastic large cell lymphoma, who usually have a good
prognosis.
ere are two types of stem cell transplants: autologous, in
which patients receive their own stem cells, and allogeneic,
in which patients receive stem cells from a matched donor.
Both types of transplants are used to treat PTCLs. e use
of autologous versus allogeneic transplant is being studied
to know what the best option is for individual patients.
Because the high-dose chemotherapy regimens used with
stem cell transplantation can cause serious side eects or
complications, including bone marrow suppression and
infections, stem cell transplants are not a treatment option
for everyone with PTCL. To determine whether you are a
good candidate for a transplant, members of your medical
team will consider your
l Medical history
l General health
l Cancer stage
l Response to previous treatment
l Age.
ere is some evidence that the use of reduced-intensity
conditioning (RIC) prior to a stem cell transplant may be
a good alternative to high-dose chemotherapy for some
PTCL patients who may be at increased risk for developing
treatment-related toxicities. However, larger studies
using RIC in PTCL patients are needed to determine the
eectiveness of the treatment. See the free LLS booklet
Blood and Marrow Stem Cell Transplantation for more
information.
Treatment Under Study
Until recently, treatment approaches for patients with
any type of PTCL were similar to treatment regimens
developed for patients with B-cell lymphomas. However,
these treatments have proven to be largely ineective for
PTCL patients. New classes of therapies that target specic
molecular pathways of T-cell lymphomas are being studied
in clinical trials. Some of the classes of novel therapies and
drugs under investigation include
l Histone deacetylase (HDAC) inhibitors, which target
“epigenetic” changes (changes that aect certain cell
processes without changing the genetic makeup of the
cell). erapies include vorinostat (Zolinza®), already
approved for the treatment of patients who have
cutaneous T-cell lymphoma, belinostat (Beleodaq™) and
panobinostat (LBH-589).
l Proteasome inhibitors, which block the action of
proteasomes. A proteasome is a large cell protein that
helps destroy other cell proteins when they are no longer
needed. Bortezomib (Velcade®), an agent that is approved
to treat patients with myeloma or mantle cell lymphoma,
is currently being evaluated in clinical trials both as
a single agent and in combination with conventional
chemotherapy.
l Immunomodulatory drugs, a novel class of small-molecule
anticancer and anti-inammatory drugs with broad
biologic activities. Lenalidomide (Revlimid®),
FDA approved to treat patients with myeloma or
myelodysplastic syndromes, is currently being studied to
treat PTCL patients.
l Monoclonal antibodies, types of protein that can bind
to tumor cells. erapies being investigated for PTCL
include alemtuzumab (Campath®), which is already
approved in the treatment of chronic lymphocytic
leukemia.
l Nucleoside analogs, which activate pathways that prevent
cell cycle progression and repair DNA. Two examples
of nucleoside analogs being studied in the treatment
of PTCL are gemcitabine (Gemzar®), approved in the
treatment of several solid tumor cancers including
ovarian and breast cancer, and nelarabine (Arranon®).
Arranon® is approved by the FDA for the treatment of
relapsed or refractory PTCL subtype precursor T-cell
acute lymphoblastic leukemia and precursor T-cell acute
lymphoblastic lymphoma in adults and children.
Treatment Outcomes
Peripheral T-cell lymphomas comprise a group of rare and
diverse diseases that are dicult to cure. As a result, standard
of care for how best to treat PTCLs in newly diagnosed
patients or in patients whose disease has relapsed (recurred)
or become refractory (does not respond to treatment) has not
been established. However, many new agents currently being
tested in clinical trials (see Treatment Under Study on this
page) are showing encouraging responses in the treatment of
PTCLs.
Because PTCLs are so rare and may be dicult to treat, it is
best to seek treatment at a medical center specializing in the
diagnosis and treatment of NHL. Your medical team will
then discuss what your treatment options are and whether
FS25 Peripheral T-Cell Lymphoma Facts I page 5
Peripheral T-Cell Lymphoma Facts
a clinical trial is right for you. If you need help locating a
medical center near you or if you need assistance nding
an appropriate clinical trial, contact an LLS Information
Specialist by calling (800) 955-4572 or by going to the LLS
website at www.LLS.org/informationspecialists.
Peripheral T-Cell Lymphoma
Subtypes
Peripheral T-Cell Lymphoma, Not Otherwise
Specied (NOS)—Peripheral T-cell lymphoma,
NOS is the most common type of PTCL and
comprises a group of mixed T-cell diseases that do
not t into any of the other subtypes of PTCL.
Most patients with PTCL-NOS will have nodal
involvement, but extranodal sites, such as the liver,
bone marrow, gastrointestinal tract and skin, may
also be involved. is group of PTCLs is considered
aggressive and in the past has usually been treated
with standard CHOP (cyclophosphamide,
hydroxydoxorubicin [doxorubicin], Oncovin®
[vincristine], prednisone) chemotherapy at initial
diagnosis. However, since the chemotherapy
combination CHOP has not produced very good
outcomes, doctors are currently evaluating other
chemotherapy combinations for initial therapy.
Other drugs that may be eective in treating
patients who have PTCL-NOS include gemcitabine
(Gemzar®) and bortezomib (Velcade®), which are
showing response rates of about 60 percent and 30
percent, respectively. A number of small studies
have demonstrated disease-free survival rates of
between 35 percent and 45 percent in some patients
who received high-dose chemotherapy followed
by autologous stem cell transplantation. However,
larger studies need to be conducted to more
accurately assess the long-term eectiveness of this
type of treatment. Clinical trials evaluating newer
combinations of therapies are ongoing.
Anaplastic Large Cell Lymphoma—is rare
T-cell lymphoma constitutes about 3 percent of
all cases of lymphomas in adults and between 10
and 30 percent of all lymphomas in children. It
usually aects nodal sites, although extranodal
sites can also be involved. Anaplastic large cell
lymphoma (ALCL) is divided into two major
subtypes based on the presence or absence of a
protein called “anaplastic lymphoma kinase (ALK).”
Patients with ALK-positive disease usually have a
good response to the chemotherapy combination
CHOP (cyclophosphamide, hydroxydoxorubicin
[doxorubicin], Oncovin® [vincristine], prednisone)
and other similar chemotherapy combinations
and can achieve long-term remission or cure.
Brentuximab vedotin, (Adcetris®) also known
as SGN-35, is FDA approved for the treatment
of patients with systemic anaplastic large cell
lymphoma (ALCL) after failure of at least one
prior multi-agent chemotherapy regimen.
Brentuximab vedotin is administered by injection.
ALK-negative patients usually relapse and may need
more aggressive treatment, including high-dose
chemotherapy and a stem cell transplant.
Anaplastic Large Cell Lymphoma (Primary
Cutaneous)—Primary cutaneous ALCL is thought
to be a more indolent (slow-growing) lymphoma and
typically aects the skin. is lymphoma is usually
ALK negative, although the prognosis is fairly good.
Angioimmunoblastic T-Cell Lymphoma—
Angioimmunoblastic T-cell lymphoma (AITL)
accounts for between 1 percent and 2 percent
of all cases of NHL and typically follows an
aggressive course, although spontaneous disease
regression sometimes occurs. AITL usually occurs
in the lymph nodes and may aect the spleen or
liver. Some symptoms may include fever, weight
loss and rashes. e chemotherapy combination
CHOP (cyclophosphamide, hydroxydoxorubicin
[doxorubicin], Oncovin® [vincristine], prednisone)
has also been commonly used for this subtype of
lymphoma, but the results do not produce many
long-term disease-free survivors. For this subtype
of lymphoma, new chemotherapy combinations
either with or without stem cell transplantation are
being evaluated. For patients with relapsed disease,
therapies such as immunosuppressive agents or
targeted agents are being evaluated in clinical trials.
Nasal, Natural Killer (NK)/T-cell Lymphoma—
Extranodal nasal, NK/T-cell lymphoma typically
aects the nasal area and the paranasal sinus
areas behind the nose and cheeks, although the
FS25 Peripheral T-Cell Lymphoma Facts I page 6
Peripheral T-Cell Lymphoma Facts
lymphoma can also occur at extranodal sites such
as the skin, gastrointestinal tract and testes. e
disease is rare in the United States, accounting
for less than 1.5 percent of all NHLs. Extranodal,
nasal NK/T-cell lymphoma is more commonly
found in Asia and Latin America and is associated
with the Epstein-Barr virus (EBV). Radiation and
chemotherapy treatments are usually recommended
for localized nasal NK/T-cell disease.
Adult T-Cell Acute Lymphoblastic Lymphoma
or Leukemia—Adult T-cell acute lymphoblastic
lymphoma or leukemia (ATLL), which is more
commonly found in Japan and the Caribbean than
in the United States, is associated with the human
T-cell leukemia virus-1 (HTLV-1). e HTLV-1
virus is transmitted through sexual intercourse,
childbirth, blood transfusions, shared needles and
breast milk. Although people who have ATLL
may initially respond to chemotherapy, the
long-term prognosis is poor. Clinical trials are
under way to investigate adding agents such as
arsenic trioxide and bortezomib (Velcade®) as well as
treatments ranging from stem cell transplantation to
conventional chemotherapy regimens.
Enteropathy-Associated Lymphoma—is T-cell
lymphoma is associated with celiac disease, a chronic
intestinal disorder caused by a hypersensitivity to
gluten proteins found in wheat, rye and barley.
Symptoms usually include stomach pain, weight
loss, gastrointestinal bleeding or bowel perforation.
Treatment for patients with enteropathy-associated
T-cell lymphoma includes an anthracycline-based
chemotherapy regimen, nutritional supplements
and, if appropriate, a gluten-free diet.
Hepatosplenic Lymphoma—is type of T-cell
lymphoma is an extremely rare and aggressive
disease that starts in the liver or spleen and usually
aects young adults in their 20s and 30s. Treatment
for patients with hepatosplenic T-cell lymphoma
includes anthracycline-based chemotherapy and, in
some cases, stem cell transplantation.
Subcutaneous Panniculitis-Like Lymphoma—
Subcutaneous panniculitis-like T-cell lymphoma
(SPTCL) is the rarest and least well-dened of the
T-cell lymphomas. is lymphoma occurs primarily
in the subcutaneous fat tissue, where it causes
nodules to form. Symptoms include fever, chills,
weight loss and oral mucosal ulcers. SPTCL may be
either rapidly aggressive or indolent (slow growing).
Treatment includes combination anthracycline-based
chemotherapy or localized radiation.
Precursor T-Cell Acute Lymphoblastic Lymphoma
or Leukemia—is subtype of PTCL may be
diagnosed as leukemia or lymphoma or both. is
cancer is found in both children and adults and is
most commonly diagnosed in adolescent and adult
males. Treatment for newly diagnosed patients with
precursor T-cell acute lymphoblastic lymphoma or
leukemia is aggressive chemotherapy and radiation.
Nelarabine (Arranon®) is approved for the treatment
of relapsed or refractory precursor T-cell acute
lymphoblastic lymphoma or leukemia in adults and
children.
Blastic NK-Cell Lymphoma—is T-cell
lymphoma is very rare, very fast growing and
dicult to treat. Patients with blastic NK-cell
lymphoma should talk to their medical team about
participating in promising clinical trials.
Acknowledgement
LLS gratefully acknowledges
Julie Vose, MD
Professor of Medicine, Division of Hematology and Oncology
e University of Nebraska Medical Center
Omaha, Nebraska
for her review of Peripheral T-Cell Lymphoma Facts and her
important contributions to the material presented in this
publication.
We’re Here to Help
LLS is the world’s largest voluntary health organization
dedicated to funding blood cancer research, education and
patient services. LLS has chapters throughout the United
States and in Canada. To nd the chapter nearest to you,
visit our website at www.LLS.org or contact
e Leukemia & Lymphoma Society
1311 Mamaroneck Avenue
Suite 310
White Plains, NY 10605
Contact an Information Specialist at (800) 955-4572
Email: [email protected]
FS25 Peripheral T-Cell Lymphoma Facts I page 7
Peripheral T-Cell Lymphoma Facts
LLS oers free information and services for patients and
families touched by blood cancers. e following lists
various resources available to you. Use this information to
learn more, to ask questions, and to make the most of your
healthcare team.
Consult with an Information Specialist. Information
Specialists are master’s level oncology social workers, nurses
and health educators. ey can answer general questions
about diagnosis and treatment options, oer guidance and
support and assist with clinical-trials searches. Language
services are available.
For more information, please
l Call: (800) 955-4572 (M-F, 9 a.m. to 9 p.m. EST)
l Email: [email protected]
l Live chat: www.LLS.org
l Visit: www.LLS.org/informationspecialists.
Free Materials. LLS oers free education and support
publications that can either be read online or downloaded.
Free print versions can be ordered.
For more information, please visit www.LLS.org/publications.
Telephone/Web Education Programs. LLS oers free
telephone/Web education programs for patients, caregivers
and healthcare professionals.
For more information, please visit www.LLS.org/programs.
Co-Pay Assistance Program. LLS oers insurance premium
and medication co-pay assistance for certain eligible patients.
For more information, please
l Call: (877) 557-2672
l Visit: www.LLS.org/copay.
Online Blood Cancer Discussion Boards and Chats.
Online discussion boards and moderated online chats can
help cancer patients to reach out, share information and
provide support.
For more information, please visit www.LLS.org/getinfo.
LLS Chapters. LLS oers support and services in the
United States and Canada including the Patti Robinson
Kaufmann First Connection Program (a peer-to-peer support
program), in-person support groups, and other great
resources.
For more information about these programs or to contact
your chapter, please
l Call: (800) 955-4572
l Visit: www.LLS.org/chapternd.
Clinical Trials (Research Studies). New treatments for
patients with PTCL are under way. Many are part of clinical
trials. Patients can learn about clinical trials and how to
access them.
For more information, please
l Call: (800) 955-4572 to speak with our LLS Information
Specialists who can help conduct clinical trial searches
l Visit: www.LLS.org/clinicaltrials.
Advocacy. e LLS Oce of Public Policy (OPP) enlists
volunteers to advocate for policies and laws to speed new
treatments and improve access to quality medical care.
For more information, please
l Call: (800) 955-4572
l Visit: www.LLS.org/advocacy.
Other Resources
e National Cancer Institute
(800) 422-6237
www.cancer.gov
e National Cancer Institute is part of the National
Institutes of Health and is a national resource center for
information and education about all forms of cancer,
including peripheral T-cell lymphomas (PTCLs). e
NCI also provides a clinical-trials search feature, the
PDQ®Cancer Clinical Trials Registry, at the website
www.cancer.gov/clinicaltrials/search-form-help, where
PTCL patients can look for clinical trials for their specic
subtype.
FocusonPTCL.org
FocusonPTCL.org (focusonptcl.org) oers comprehensive
information on PTCLs, including treatment options.
References
Chen AI, Advani RH. Beyond the guidelines in the
treatment of peripheral T-cell lymphoma: new drug
development. Journal of the National Comprehensive Cancer
Network. 2008;6(4):428-435.
Foss F. Evolving therapy of peripheral T-cell lymphoma.
erapeutic Advances in Hematology. 2011;2(3):161-173.
Foss FM, Zinzani PL, Vose JM, et al. Peripheral T-cell
lymphoma. Blood. 2011;117(25):6756-6767.
FS25 Peripheral T-Cell Lymphoma Facts I page 8
Peripheral T-Cell Lymphoma Facts
Gallamini A, Stelitano C, Calvi R et al. Peripheral T-cell
lymphoma unspecied (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
(specied and unspecied 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.
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