Peripheral T-cell lymphoma, not otherwise specified

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Peripheral T-cell lymphoma, not otherwise specified
This fact sheet is about a type of
non-Hodgkin lymphoma known
as peripheral T-cell lymphoma, not
otherwise specified. It should ideally be
read with our general information about
non-Hodgkin lymphoma.
We hope this fact sheet answers any
questions you may have. If you have any
further questions, you can ask your doctor
or nurse at the hospital where you are
having your treatment.
If you’d like to discuss this information
with our cancer support specialists,
call the Macmillan Support Line free on
0808 808 00 00, Monday–Friday,
9am–8pm. If you’re hard of hearing you
can use textphone 0808 808 0121,
or Text Relay. For non-English speakers,
interpreters are available. Alternatively,
Includes the following information
• Non-Hodgkin lymphoma
• Peripheral T-cell lymphomas
• Peripheral T-cell lymphoma,
not otherwise specified
• Causes
• Signs and symptoms
• How it is diagnosed
• Staging and grading
• Treatment
• Follow-up
• Clinical trials
• Information and support
• Other useful organisations
• Related Macmillan information
Non-Hodgkin lymphoma
Non-Hodgkin lymphoma (NHL) is a
cancer of the lymphatic system. The
lymphatic system is part of the body’s
immune system and helps us fight
infection. It’s made up of organs such
as the bone marrow, thymus, spleen, and
lymph nodes (or lymph glands). There is
also lymphatic tissue in other organs such
as the lungs, stomach and skin.
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Neck (cervical)
lymph nodes
Thymus gland
Armpit (axillary)
lymph nodes
Groin (inguinal)
lymph nodes
The lymphatic system
There are lymph nodes all over the body.
They are connected by a network of tiny
lymphatic vessels that contain a fluid called
lymph. As lymph fluid flows through the
lymph nodes, the nodes collect and filter
out anything that the body doesn’t need,
or that could harm the body. This includes
bacteria, viruses, damaged cells, and
cancer cells.
Lymph contains cells called lymphocytes.
Lymphocytes are a type of white blood cell
and are an essential part of the body’s
defence against infection and disease.
Most lymphocytes start growing in the bone
marrow, which is where blood cells are
made. The two main types of lymphocytes
are B-cells and T-cells. The B-cells continue
to develop in the bone marrow. The T-cells
go from the bone marrow to the thymus
gland (behind the breast bone) and mature
there. When they are mature, both B-cells
and T-cells help us fight infections.
Lymphoma is a disease in which either
T- or B-cells grow in an uncontrolled way.
There are many different types of nonHodgkin lymphoma. They are grouped
(or classified) according to certain
characteristics. The most widely used
classification system is produced by the
World Health Organisation (WHO).
Peripheral T-cell lymphomas
Peripheral T-cell lymphomas (PTCL) are a
type of NHL that make up less than 10%
(1 in 10) of all cases of NHL in adults.
There are different sub-types of PTCL
depending on the characteristics of the
The different sub-groups include:
• peripheral T-cell lymphoma,
not otherwise specified
• anaplastic large cell lymphoma
• angioimmunoblastic T-cell lymphoma
• enteropathy-associated T cell lymphoma
• some very rare types of PTCL.
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We have more detailed information about
anaplastic large cell lymphoma. For other
rare types of lymphoma, contact our cancer
support specialists on freephone 0808 808
00 00. They can give you more information
about your illness and its treatment.
Peripheral T-cell lymphoma,
not otherwise specified
Peripheral T-cell lymphoma, not otherwise
specified (PTCL, NOS) is a sub-type that
is made up of all the peripheral T-cell
lymphomas that don’t fit into any other
sub-type and are therefore ‘not otherwise
specified’ or ‘unspecified’. As scientists
and doctors discover more about the
different types of PTCL within this subgroup, it’s likely that further sub-groups
will be identified.
PTCL, NOS can occur at any age but it’s
more common in adults. It affects both
men and women equally.
The causes of PTCL, NOS are unknown.
It is not infectious and can’t be passed on
to other people.
Signs and symptoms
The first signs of lymphoma vary, but
it’s often a painless swelling in the neck,
armpit, or groin, caused by enlarged lymph
nodes. Lymph nodes in several different
areas are frequently involved. Some people
experience a loss of appetite and tiredness.
A lymphoma can also affect other organs
in the body such as the bone marrow, liver,
spleen, stomach, bowel or the skin. It’s
fairly common for PTCL, NOS to affect the
bone marrow, liver and skin.
Other symptoms of lymphoma may
include night sweats, unexplained high
temperatures and weight loss. These are
known as B symptoms and are fairly
common with PTCL, NOS.
How it is diagnosed
The doctors at the hospital will remove an
enlarged lymph node or part of it, and
examine the cells under a microscope.
This is called a biopsy. It’s a very small
operation and may be done under local
or general anaesthetic. You may also have
biopsies taken from other parts of the body.
Further tests – including blood tests, x-rays,
scans, and bone-marrow samples – are
then used to get more information about
the type of lymphoma and how far it has
spread in the body. This information is
used to help decide which treatment is
most appropriate for you. There's more
information about the tests that may be
used in our booklet Understanding nonHodgkin lymphoma.
Staging and grading
The stage of NHL describes how many
groups of lymph nodes are affected, where
they are in the body, and whether other
organs such as the bone marrow or liver
are affected.
Stage 1 The lymphoma is only in one
group of lymph nodes, in one particular
area of the body.
Stage 2 More than one group of lymph
nodes is affected, but all the affected nodes
are in either the upper half or the lower
half of the body. The upper half of the
body is above the diaphragm (the sheet
of muscle underneath the lungs), and the
lower half is below it.
Stage 3 Lymphoma is present in lymph
nodes in both the upper and the lower
parts of the body (ie in lymph nodes both
above and below the diaphragm). The
spleen is considered as a lymph node in
this staging system.
Stage 4 The lymphoma has spread beyond
the lymph nodes to other lymphatic organs,
for example to sites such as the bone
marrow, liver or lungs.
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The stage usually includes the letter A or
B, which describes whether B symptoms
are present or not (eg stage 2B). Sometimes
a lymphoma can start in areas outside the
lymph nodes, and this is represented by
the letter E, which stands for extranodal
(eg stage 3AE).
For practical purposes, non-Hodgkin
lymphomas are also divided into two
groups, low- and high-grade. Low-grade
lymphomas are usually slow-growing,
and high-grade lymphomas tend to grow
more quickly.
Peripheral T-cell lymphoma behaves as a
high-grade lymphoma and usually needs
prompt treatment.
Chemotherapy is the main treatment
for PTCL, NOS. Some people may also
have stem cell treatments (see below).
Radiotherapy is occasionally given after
chemotherapy to treat early-stage disease
(stages 1 and 2), or sometimes to help
control symptoms (palliative radiotherapy).
PTCL, NOS can be quite difficult to
treat successfully. Some people are
cured by chemotherapy, but usually the
chemotherapy will reduce and control the
condition for a period of time (remission).
The risk of the PTCL, NOS coming back
after initial treatment is quite high. If
this happens, further chemotherapy can
sometimes be given to try to control it
again, but this isn’t always possible.
Chemotherapy is the use of anti-cancer
(cytotoxic) drugs to destroy cancer cells.
A combination of chemotherapy drugs
is usually used. Different combinations
can be used to treat PTCL, and the best
combination isn’t known. You may be
asked to take part in a clinical trial (see
page 5) that’s trying to find out which is
the best to use.
The most commonly used chemotherapy
combination is known as CHOP. It
includes the chemotherapy drugs
vincristine, doxorubicin, cyclophosphamide
and the steroid prednisolone.
If the lymphoma doesn’t respond to CHOP
or comes back at a later date, other
chemotherapy combinations may be used.
We have a fact sheet on CHOP
chemotherapy that you may find useful.
High-dose treatment with stem
cell support
High-dose treatment with stem cell support
(autologous transplant) is sometimes
used for this type of lymphoma. It may
be offered to some people following
their initial course of chemotherapy. It
can sometimes be used if the lymphoma
comes back, where it is again used after
chemotherapy has been given.
High-dose treatment with stem cell support
involves having intensive chemotherapy,
which destroys the lymphoma cells but
also destroys the blood cells in your
bone marrow. Before you have the
chemotherapy, you'll have some of your
own stem cells (early blood cells) taken and
stored. These are given back to you (like a
blood transfusion) after the chemotherapy.
The stem cells make their way back to your
bone marrow and start to produce new
blood cells.
Your doctor will take into account your age
and general health before recommending
this treatment. This is because the intensity
of treatment increases the risks of serious
side effects.
Some people with PTCL, NOS may have
treatment using stem cells from another
person (a donor). This is called an
allogeneic transplant and is mainly used if
the lymphoma comes back (relapses) after
initial treatment.
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We have further information about these
treatments in our booklets Understanding
high-dose treatment with stem cell support
and Understanding donor stem cell
(allogeneic) transplants.
Radiotherapy uses high-energy rays
to destroy cancer cells, while doing as
little harm as possible to healthy cells.
It‘s sometimes used after chemotherapy
for people whose lymphoma cells are
contained in one area of the body
(stage 1 or 2). Radiotherapy is also
sometimes used to control symptoms
(palliative radiotherapy).
Steroid therapy
Steroids are drugs that are often given with
chemotherapy to help treat lymphomas.
They also help you feel better and can
reduce feelings of sickness (nausea).
After your treatment has been completed,
your doctor will ask you to go back
to hospital for regular check-ups. You
will usually have a scan at the end of
treatment to assess how well the treatment
has worked. These scans aren’t usually
carried out if you feel well. Your followup appointments are good opportunities
to discuss with your doctor any worries or
problems you may have. However, if you
notice any new symptoms or are anxious
about anything else between appointments,
contact your doctor or nurse for advice.
Clinical trials
New treatments for PTCL, NOS are being
researched all the time. You may be invited
by your doctor to take part in a clinical trial
to compare a new treatment with the best
available standard treatment. Your doctor
must discuss the treatment with you, and
have your consent before entering you into
any clinical trial.
Before any trial is allowed to take place,
it must be approved by a research ethics
committee, which protects the interests of
those taking part.
You may decide not to take part or to
withdraw from the trial at any stage.
You will then receive the best standard
treatment available.
Newer treatments for PTCL, NOS that are
being researched in clinical trials include
the chemotherapy drug gemcitabine and
the biological therapy alemtuzumab.
Information and support
Everyone has their own way of dealing with
their illness and the different emotions they
experience. You may find it helpful to talk
things over with your family and friends
or with your doctor or nurse. You can
also contact our cancer support specialists
on freephone 0808 808 00 00 or the
organisations below for more information
and support.
Other useful organisations
Leukaemia & Lymphoma Research
43 Great Ormond Street,
London WC1N 3JJ
Tel 020 7405 0101
Email [email protected]
Provides information on non-Hodgkin
lymphoma and other blood disorders.
Leukaemia Care
1 Birch Court, Blackpole East,
Worcester WR3 8SG
Tel 0800 169 6680 (24 hour)
Email [email protected]
A national group promoting the welfare
of people with leukaemia and related
blood disorders, including non-Hodgkin
lymphoma. Has regional support groups
in many counties.
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The Lymphoma Association
PO Box 386, Aylesbury HP20 2GA
Tel 0808 808 5555
Email [email protected]
Provides information and emotional support
to people with lymphoma and
their families, carers and friends.
Related Macmillan information
• CHOP chemotherapy
• Steroids
• The emotional effects of cancer
• Understanding donor stem cell
(allogeneic) transplants
This fact sheet has been compiled using information
from a number of reliable sources, including:
Freedman AS et al. Clinical manifestations,
pathologic features, and diagnosis of peripheral
T-cell lymphoma, not otherwise specified.
UpToDate Online, 2011. (accessed July 2011).
Guidelines for the management of mature T-cell
and NK-cell neoplasms (excluding cutaneous
T-cell lymphoma). British Committee for
Standards in Haematology (BCSH), 2011.
Jacobson E et al. Initial treatment of peripheral
T-cell lymphoma. UpToDate Online, 2011.
(accessed July 2011).
Lee et al (eds). Wintrobe’s Clinical Haematology
(12th edition). 2008. Williams and Wilkins.
Improving outcomes in haemato-oncology.
November 2003. National Institute of Clinical
Excellence (NICE).
• Understanding cancer research trials
• Understanding chemotherapy
This fact sheet was revised in 2012. The next edition
will be available in 2013.
• Understanding high-dose treatment
with stem cell support
• Understanding non-Hodgkin lymphoma
• Understanding radiotherapy
For copies of this related information call
free on 0808 808 00 00, or see it online
This fact sheet has been written, revised and edited
by Macmillan Cancer Support’s Cancer Information
Development team. It has been approved by our
medical editor, Dr Terry Priestman, Consultant
Clinical Oncologist.
With thanks to Professor Rajnish Gupta, Consultant
Oncologist; Dr Graham Jackson, Consultant
Haematologist; Dr Pam McKay, Lymphoma Lead
Clinician; and the people affected by cancer who
reviewed this edition.
We make every effort to ensure that the
information we provide is accurate but it should
not be relied upon to reflect the current state of
medical research, which is constantly changing.
If you are concerned about your health, you
should consult your doctor. Macmillan cannot
accept liability for any loss or damage resulting
from any inaccuracy in this information or
third-party information, such as information on
websites to which we link.
© Macmillan Cancer Support 2012. Registered
charity in England and Wales (261017),
Scotland (SC039907) and the Isle of Man
(604). Registered office 89 Albert Embankment,
London, SE1 7UQ.
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