Understanding the Subtypes of Peripheral
T-Cell Lymphoma (PTCL)
Understanding the Subtypes of Peripheral
T-Cell Lymphoma (PTCL)
Understanding the Subtypes of Peripheral
T-Cell Lymphoma (PTCL)
Although they share the name PTCL, not all peripheral T-cell lymphomas are the same. There are
several different subtypes, each with unique features and treatment approaches. Identifying
your exact subtype is important because it helps your doctor create the treatment plan that is
most effective for you.
Some of the more common subtypes include:
Although they share the name PTCL, not all peripheral T-
cell lymphomas are the same. There are several different
subtypes, each with unique features and treatment
approaches. Identifying your exact subtype is important
because it helps your doctor create the treatment plan
that is most effective for you.
Some of the more common subtypes include:
Anaplastic Large Cell Lymphoma (ALCL)
ALCL is a type of T-cell lymphoma that can manifest in various forms, depending on the part of the
body it affects and whether the cancer cells express a protein called ALK (anaplastic lymphoma
kinase).
The first important distinction is between systemic ALCL and primary cutaneous ALCL:
Systemic ALCL affects lymph nodes or other parts of the body beyond the skin. These lymphomas
can grow quickly, often needing urgent treatment. Systemic ALCL can be further divided by ALK
status:ALK-positive ALCL usually occurs in younger people, often responds well to treatment, and
generally has a better outlook.ALK-negative ALCL tends to occur in older adults and can be more aggressive, often coming back
after initial treatment.
Primary cutaneous ALCL mostly affects the skin. It may cause red, raised, or ulcerated patches or
lumps, but usually stays in the skin and rarely spreads. This type usually grows slowly, and in some
cases, the skin lesions can stay for a long time or grow gradually over months or years.
ALCL is a type of T-cell lymphoma that can manifest in various forms, depending on the part of the
body it affects and whether the cancer cells express a protein called ALK (anaplastic lymphoma
kinase).
The first important distinction is between systemic ALCL and primary cutaneous ALCL:
Systemic ALCL affects lymph nodes or other parts of the body beyond the skin. These
lymphomas can grow quickly, often needing urgent treatment. Systemic ALCL can be further
divided by ALK status:ALK-positive ALCL usually occurs in younger people, often responds well to treatment, and
generally has a better outlook.ALK-negative ALCL tends to occur in older adults and can be more aggressive, often coming
back after initial treatment.
Primary cutaneous ALCL mostly affects the skin. It may cause red, raised, or ulcerated patches
or lumps, but usually stays in the skin and rarely spreads. This type usually grows slowly, and in
some cases, the skin lesions can stay for a long time or grow gradually over months or years.
ALCL is a type of T-cell lymphoma that can manifest in
various forms, depending on the part of the body it
affects and whether the cancer cells express a protein
called ALK (anaplastic lymphoma kinase).
The first important distinction is between systemic ALCL
and primary cutaneous ALCL:
Systemic ALCL affects lymph nodes or other parts of
the body beyond the skin. These lymphomas
can grow quickly, often needing urgent treatment.
Systemic ALCL can be further divided by ALK
status:ALK-positive ALCL usually occurs in younger
people, often responds well to treatment, and
generally has a better outlook.ALK-negative ALCL tends to occur in older adults
and can be more aggressive, often coming back
after initial treatment.
Primary cutaneous ALCL mostly affects the skin. It may
cause red, raised, or ulcerated patches or lumps, but
usually stays in the skin and rarely spreads. This type
usually grows slowly, and in some cases, the skin
lesions can stay for a long time or grow gradually over
months or years.
T-Follicular Helper (TFH) Cell Lymphoma
T-Follicular Helper (TFH) Cell
Lymphoma
T-follicular helper (TFH) cell lymphoma is a type of PTCL that starts in special immune cells called TFH
cells, which normally help the body make antibodies to fight infections. This lymphoma usually affects
the lymph nodes but can sometimes involve other parts of the body. It often “turns on” the immune
system and causes inflammation, and because it can be aggressive, treatment usually needs to start
quickly. TFH cell lymphomas often have changes in the “genetic switches” that control how cells
behave, called epigenetic mutations.
Common signs and symptoms include swollen lymph nodes, fevers, night sweats, weight loss, skin
rashes, joint pain, low blood counts, and breakdown of red blood cells. Some cases may also be linked
to the Epstein-Barr virus (EBV) in nearby immune cells.
T-follicular helper (TFH) cell lymphoma is a type of PTCL that starts in special immune cells called
TFH cells, which normally help the body make antibodies to fight infections. This lymphoma usually
affects the lymph nodes but can sometimes involve other parts of the body. It often “turns on” the
immune system and causes inflammation, and because it can be aggressive, treatment usually
needs to start quickly. TFH cell lymphomas often have changes in the “genetic switches” that
control how cells behave, called epigenetic mutations.
Common signs and symptoms include swollen lymph nodes, fevers, night sweats, weight loss, skin
rashes, joint pain, low blood counts, and breakdown of red blood cells. Some cases may also be
linked to the Epstein-Barr virus (EBV) in nearby immune cells.
T-follicular helper (TFH) cell lymphoma is a type of PTCL
that starts in special immune cells called TFH cells, which
normally help the body make antibodies to fight
infections. This lymphoma usually affects the lymph
nodes but can sometimes involve other parts of the body.
It often “turns on” the immune system and causes
inflammation, and because it can be aggressive,
treatment usually needs to start quickly. TFH cell
lymphomas often have changes in the “genetic switches”
that control how cells behave, called epigenetic
mutations.
Common signs and symptoms include swollen lymph nodes, fevers, night sweats, weight loss, skin rashes, joint
pain, low blood counts, and breakdown of red blood cells.
Some cases may also be linked to the Epstein-Barr virus (EBV) in nearby immune cells.
Peripheral T-Cell Lymphoma – Not Otherwise Specified (PTCL-NOS)
Peripheral T-Cell Lymphoma –
Not Otherwise Specified (PTCL-NOS)
PTCL-NOS is the most common type of PTCL, but it can be harder to describe. It’s sometimes called
the “catch-all” category because it includes PTCL that don’t fit into other specific groups. This type
usually grows quickly, and treatment is tailored to each person because it can behave differently in
different people.
PTCL-NOS is the most common type of PTCL, but it can be harder to describe. It’s sometimes
called the “catch-all” category because it includes PTCL that don’t fit into other specific groups.
This type usually grows quickly, and treatment is tailored to each person because it can behave
differently in different people.
PTCL-NOS is the most common type of PTCL, but it can
be harder to describe. It’s sometimes called the “catch-
all” category because it includes PTCL that don’t fit into
other specific groups. This type usually grows quickly,
and treatment is tailored to each person because it can
behave differently in different people.
Adult T-Cell Leukemia/Lymphoma (ATLL)
Adult T-cell leukemia/lymphoma (ATLL) is a rare PTCL subtype caused by a virus called HTLV-1 (Human
T-cell Leukemia Virus Type 1). This virus can be passed from person to person through blood, sexual
contact, or from mother to baby during breastfeeding. ATLL is most common in certain parts of the
world, including Japan, the Caribbean, South America, and some areas of Africa.
ATLL can look different in different people. Some people have very high white blood cell counts and an
enlarged liver or spleen. Others may have swollen lymph nodes or skin rashes and bumps. ATLL can
grow quickly and may affect the blood, skin, and lymph nodes at the same time.
Adult T-cell leukemia/lymphoma (ATLL) is a rare PTCL subtype caused by a virus called HTLV-1
(Human T-cell Leukemia Virus Type 1). This virus can be passed from person to person through
blood, sexual contact, or from mother to baby during breastfeeding. ATLL is most common in
certain parts of the world, including Japan, the Caribbean, South America, and some areas of
Africa.
ATLL can look different in different people. Some people have very high white blood cell counts and
an enlarged liver or spleen. Others may have swollen lymph nodes or skin rashes and bumps. ATLL
can grow quickly and may affect the blood, skin, and lymph nodes at the same time.
Adult T-cell leukemia/lymphoma (ATLL) is a rare PTCL subtype caused by a virus called HTLV-1 (Human
T-cell Leukemia Virus Type 1). This virus can be passed
from person to person through blood, sexual contact, or
from mother to baby during breastfeeding. ATLL is most
common in certain parts of the world, including Japan,
the Caribbean, South America, and some areas of Africa.
ATLL can look different in different people. Some people
have very high white blood cell counts and an enlarged
liver or spleen. Others may have swollen lymph nodes or
skin rashes and bumps. ATLL can grow quickly and may
affect the blood, skin, and lymph nodes at the same time.
Extranodal NK/T-Cell Lymphoma (ENKTL), Nasal Type
Extranodal NK/T-Cell
Lymphoma (ENKTL), Nasal Type
Extranodal NK/T-cell lymphoma (ENKTL) is a rare subtype of PTCL that usually grows and spreads
quickly. The word “extranodal” means it starts outside of the lymph nodes, most often in the nasal
passages or upper airway. This can cause symptoms such as a blocked nose, frequent nosebleeds,
facial swelling, or pain. Some people may notice sinus problems that don’t improve with usual
treatments.
ENKTL is more common in parts of Asia and South America and is strongly linked to infection with the
Epstein-Barr virus (EBV). Doctors can often find EBV in the cancer cells or blood, which can help with
diagnosis and monitoring.
Extranodal NK/T-cell lymphoma (ENKTL) is a rare subtype of PTCL that usually grows and spreads
quickly. The word “extranodal” means it starts outside of the lymph nodes, most often in the nasal
passages or upper airway. This can cause symptoms such as a blocked nose, frequent nosebleeds,
facial swelling, or pain. Some people may notice sinus problems that don’t improve with usual
treatments.
ENKTL is more common in parts of Asia and South America and is strongly linked to infection with
the Epstein-Barr virus (EBV). Doctors can often find EBV in the cancer cells or blood, which can
help with diagnosis and monitoring.
Extranodal NK/T-cell lymphoma (ENKTL) is a rare subtype of PTCL that usually grows and spreads quickly. The word
“extranodal” means it starts outside of the lymph nodes,
most often in the nasal passages or upper airway. This
can cause symptoms such as a blocked nose, frequent
nosebleeds, facial swelling, or pain. Some people may
notice sinus problems that don’t improve with usual
treatments.
ENKTL is more common in parts of Asia and South
America and is strongly linked to infection with the
Epstein-Barr virus (EBV). Doctors can often find EBV in
the cancer cells or blood, which can help with diagnosis
and monitoring.
T-cell prolymphocytic leukemia (T-PLL)
T-cell prolymphocytic leukemia (T-PLL) is another rare subtype of PTCL. Unlike most other T-cell
cancers that begin in the lymph nodes or organs, T-PLL mainly grows in the blood and bone marrow.
T-PLL can start slowly but may become more aggressive over time. Many people are diagnosed when
they have very high white blood cell counts, an enlarged spleen, or symptoms such as feeling very
tired, losing weight without trying, night sweats, frequent infections, or easy bruising. T-PLL cells can
also spread to the liver, lymph nodes, skin, or the fluid around the lungs, which can cause skin rashes,
swelling, or difficulty breathing.
T-cell prolymphocytic leukemia (T-PLL) is another rare subtype of PTCL. Unlike most other T-cell
cancers that begin in the lymph nodes or organs, T-PLL mainly grows in the blood and bone marrow.
T-PLL can start slowly but may become more aggressive over time. Many people are diagnosed
when they have very high white blood cell counts, an enlarged spleen, or symptoms such as feeling
very tired, losing weight without trying, night sweats, frequent infections, or easy bruising. T-PLL
cells can also spread to the liver, lymph nodes, skin, or the fluid around the lungs, which can cause
skin rashes, swelling, or difficulty breathing.
T-cell prolymphocytic leukemia (T-PLL) is another rare
subtype of PTCL. Unlike most other T-cell cancers that
begin in the lymph nodes or organs, T-PLL mainly grows
in the blood and bone marrow.
T-PLL can start slowly but may become more aggressive
over time. Many people are diagnosed when they have
very high white blood cell counts, an enlarged spleen, or
symptoms such as feeling very tired, losing weight
without trying, night sweats, frequent infections, or easy
bruising. T-PLL cells can also spread to the liver, lymph
nodes, skin, or the fluid around the lungs, which can
cause skin rashes, swelling, or difficulty breathing.
Large Granular Lymphocyte (LGL) leukemia
Large Granular Lymphocyte
(LGL) leukemia
T-cell large granular lymphocytic leukemia (T-LGLL) is a type of blood cancer that usually shows up in
the blood rather than in the lymph nodes or organs. Unlike most other T-cell cancers, T-LGLL grows
slowly and often does not cause problems right away. It is often linked to autoimmune conditions,
where the immune system attacks the body’s own cells. T-LGLL usually affects older adults and is often
found by accident during routine blood tests.
A key sign of T-LGLL is having high numbers of T-cells in the blood for more than six months. Many
people also have low blood counts or an enlarged spleen, which may cause fatigue or a feeling of
fullness. Because it grows slowly, doctors often start with careful observation instead of immediate
treatment.
T-cell large granular lymphocytic leukemia (T-LGLL) is a type of blood cancer that usually shows up
in the blood rather than in the lymph nodes or organs. Unlike most other T-cell cancers, T-LGLL
grows slowly and often does not cause problems right away. It is often linked to autoimmune
conditions, where the immune system attacks the body’s own cells. T-LGLL usually affects older
adults and is often found by accident during routine blood tests.
A key sign of T-LGLL is having high numbers of T-cells in the blood for more than six months. Many
people also have low blood counts or an enlarged spleen, which may cause fatigue or a feeling of
fullness. Because it grows slowly, doctors often start with careful observation instead of immediate
treatment.
T-cell large granular lymphocytic leukemia (T-LGLL) is a
type of blood cancer that usually shows up in the blood
rather than in the lymph nodes or organs. Unlike most
other T-cell cancers, T-LGLL grows slowly and often does
not cause problems right away. It is often linked to
autoimmune conditions, where the immune system
attacks the body’s own cells. T-LGLL usually affects older
adults and is often found by accident during routine blood
tests.
A key sign of T-LGLL is having high numbers of T-cells in
the blood for more than six months. Many people also
have low blood counts or an enlarged spleen, which may
cause fatigue or a feeling of fullness. Because it grows
slowly, doctors often start with careful observation
instead of immediate treatment.
Enteropathy-Associated T-Cell Lymphoma (EATL)
Enteropathy-associated T-cell lymphoma (EATL) is a rare subtype of PTCL that usually affects people
with celiac disease, an autoimmune condition caused by gluten in the diet. In celiac disease, long-term
inflammation in the gut can sometimes lead to the growth of lymphoma cells.
Common symptoms include ongoing stomach pain, diarrhea, unexplained weight loss, or sudden bowel
blockage or tears. Because these symptoms can be mild or vague, EATL is sometimes diagnosed later
than other cancers.
Enteropathy-associated T-cell lymphoma (EATL) is a rare subtype of PTCL that usually affects
people with celiac disease, an autoimmune condition caused by gluten in the diet. In celiac disease,
long-term inflammation in the gut can sometimes lead to the growth of lymphoma cells.
Common symptoms include ongoing stomach pain, diarrhea, unexplained weight loss, or sudden
bowel blockage or tears. Because these symptoms can be mild or vague, EATL is sometimes
diagnosed later than other cancers.
Enteropathy-associated T-cell lymphoma (EATL) is a rare
subtype of PTCL that usually affects people with celiac
disease, an autoimmune condition caused by gluten in the
diet. In celiac disease, long-term inflammation in the gut
can sometimes lead to the growth of lymphoma cells.
Common symptoms include ongoing stomach pain,
diarrhea, unexplained weight loss, or sudden bowel
blockage or tears. Because these symptoms can be mild
or vague, EATL is sometimes diagnosed later than other cancers.
Hepatosplenic T-Cell Lymphoma (HSTCL)
Hepatosplenic T-cell lymphoma (HSTCL) is a very rare type of T-cell lymphoma and is considered the
most aggressive subtype of PTCL. It often affects young adults, especially those with weakened
immune systems, such as people who have had an organ transplant or are on long-term
immunosuppressive therapy.
HSTCL mainly involves the liver, spleen, and bone marrow, and usually spares the lymph nodes.
Common symptoms can include fever, unexplained weight loss, fatigue, night sweats, and an enlarged
liver or spleen.
Hepatosplenic T-cell lymphoma (HSTCL) is a very rare type of T-cell lymphoma and is considered
the most aggressive subtype of PTCL. It often affects young adults, especially those with weakened
immune systems, such as people who have had an organ transplant or are on long-term
immunosuppressive therapy.
HSTCL mainly involves the liver, spleen, and bone marrow, and usually spares the lymph nodes.
Common symptoms can include fever, unexplained weight loss, fatigue, night sweats, and an
enlarged liver or spleen.
Hepatosplenic T-cell lymphoma (HSTCL) is a very rare
type of T-cell lymphoma and is considered the most
aggressive subtype of PTCL. It often affects young adults,
especially those with weakened immune systems, such
as people who have had an organ transplant or are on
long-term immunosuppressive therapy.
HSTCL mainly involves the liver, spleen, and bone
marrow, and usually spares the lymph nodes. Common
symptoms can include fever, unexplained weight loss,
fatigue, night sweats, and an enlarged liver or spleen.
Monomorphic epitheliotropic intestinal T-Cell lymphoma (MEITL)
Monomorphic epitheliotropic
intestinal T-Cell lymphoma (MEITL)
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive type of T-cell
lymphoma that affects the small intestine. The term “epitheliotropic” means that the cancer cells stick to
the lining of the gut. It is more common in adults and can cause symptoms such as abdominal pain,
diarrhea, weight loss, or intestinal blockage.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive type of T-
cell lymphoma that affects the small intestine. The term “epitheliotropic” means that the cancer cells
stick to the lining of the gut. It is more common in adults and can cause symptoms such as
abdominal pain, diarrhea, weight loss, or intestinal blockage.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive type of T-cell lymphoma that affects the small intestine. The term “epitheliotropic” means that the cancer cells stick to the lining of the gut. It is more common in adults and can cause symptoms such as abdominal pain, diarrhea, weight loss, or intestinal blockage.
Why Subtypes Matter
Even though these subtypes all fall under “PTCL,” they are biologically different. Your doctor uses information from your biopsy, lab tests, and molecular studies to figure out your exact subtype. This helps guide decisions about:
Treatment options
Clinical trial eligibility
Chances of response or remission
Even though these subtypes all fall under “PTCL,” they are biologically different. Your doctor uses
information from your biopsy, lab tests, and
molecular studies to figure out your exact subtype. This helps guide decisions about:
Treatment options
Clinical trial eligibility
Chances of response or remission
Treatment options, prognosis (expected progression of disease), and risks differ widely by subtype. Knowing exactly which type is the key to appropriate treatment approach.
Understanding your symptoms can help; Helps link what you are feeling to your disease location.your disease location to what you may be experiencing
In Summary
Peripheral T-cell lymphoma isn’t one disease—it’s a group of rare and complex cancers, each with
its own challenges.
Understanding your subtype helps you and your healthcare team make informed choices about
treatment and next steps.If you’re unsure about your subtype, don’t hesitate to ask your doctor—it’s a key part of your care
plan.
Peripheral T-cell lymphoma isn’t one disease—it’s a group of rare and complex cancers, each
with its own challenges.
Understanding your subtype helps you and your healthcare team make informed choices about
treatment and next steps.If you’re unsure about your subtype, don’t hesitate to ask your doctor—it’s a key part of your care
plan.
Related Reference
Related Reference
Diagnostic Frontiers: Histopathology & Classification of Peripheral T-Cell Lymphomas
Murga-Zamalloa C, Inamdar K. Frontiers | Classification and challenges in the histopathological diagnosis of peripheral T-cell lymphomas, emphasis on the WHO-HAEM5 updates. Frontiers in Oncology. 2022/12/20;12.