Peripheral T-Cell Lymphomas (PTCLs)
PTCLs are a group of aggressive
lymphomas that affect a certain type of T-cell. They account for approximately
7% of NHL cases. There are many sub-types of PTCLs, including:
Subcutaneous panniculitis-like T-cell lymphoma:
This type of PTCL is quite rare and is often
confused with a condition called panniculitis, an inflammation of fatty tissue
in the body. The most common symptoms include nodules under the skin
(subcutaneous nodules) which can progress to open, inflamed sores.
Haemophagocytic syndrome—a serious condition in which there is uncontrolled
activation of certain parts of the immune system— is also common in this
Hepatosplenic gamma delta T-cell lymphoma:
This type of PTCL affects the whole body, with
infiltration of the liver, spleen and bone marrow by T-cells. Usually
there are no actual tumours. It is associated with systemic symptoms including
fever, weight loss, night sweats, fatigue and is quite difficult to diagnose.
Enteropathy-type intestinal T-cell lymphoma:
This type of PTCL is very rare and occurs in people
with untreated gluten-sensitive intestinal disease, called celiac disease.
These people are often in a very weakened state and may have intestinal
perforation (an abnormal hole in the wall of the intestine) when they are
Extranodal T-cell lymphoma, nasal type:
This type of PTCL, previously referred to as
angiocentric lymphoma, is more common in Asia and South America. It most
frequently affects the nose and nasal passages but can involve other organs as
well. It has an aggressive course, and haemophagocytic syndrome can also occur
in this condition.
Angioimmunoblastic T-cell lymphoma:
This is a more common sub-type of PTCL, accounting
for approximately 20% of all T-cell lymphomas. Symptoms include generalised
lymphadenopathy (swollen lymph nodes), fever, weight loss, skin rash and high
levels of antibodies in the blood.
PTCL, unspecified. This is the most common PTCL
sub-type. It represents all of the PTCLs lacking in a clear definition and thus
not classifiable as a specific sub-type. Most people with PTCL, unspecified
have lymph node involvement however a number of extranodal sites may also be
involved such as liver, bone marrow, intestinal tract and skin.
How are they treated?
Treatment of PTCLs are similar to those used for
other aggressive lymphomas, such as combination chemotherapy medicines,
radiotherapy, targeted therapies and stem-cell transplant when required. The
response to treatment is not often as effective in PTCLs as it is in DLBCL. and
as a result, stem-cell transplantation is sometimes considered an early
treatment option in appropriate cases.
Acute Lymphoblastic Leukaemia/Lymphoma (T-ALL)
T-cell Acute Lymphoblastic Lymphoma (T-ALL)
s a type of aggressive lymphoma that occurs mainly in children and adolescents
and more often in males than females. A second peak of occurrence is seen later
in life in people over 40 years of age. Lymphoblastic cancers are classified as
either lymphoblastic leukaemia’s or lymphoblastic lymphomas.
The most common symptoms include
breathing difficulties and other problems resulting from a large mass in the
mediastinal area (the centre area of the upper chest), as well as fluid
accumulation around the lungs. This type of lymphoma can spread to the central
nervous system and neurological symptoms may also be present at diagnosis.
How is it treated?
Intensive combination chemotherapy
medicines are the most common treatment for older children and young adults
with aggressive lymphoblastic lymphoma. Young people with localised disease
have an excellent prognosis. Adults with later stage disease may have a stem
cell transplant as part of their initial treatment plan.