What is PTCL cancer?
PTCL is a rare and often fast-growing cancer that develops from white blood cells called T-lymphocytes, or T-cells. T-cells are an important part of the immune system; they help your body fight infection. In cases when some T-cells start to grow too quickly and out of control, they can accumulate in the body.
Can PTCL-NOS be cured?
PTCL is potentially curable with chemotherapy. The median progression free survival (PFS) following CHOP chemotherapy is 12 to 14 months. Approximately 20 to 30% of patients will be alive, and disease free, 5 years after the completion of treatment.
What does lymphoma Nos mean?
When a T-cell lymphoma doesn’t fit into any particular category of T-cell lymphoma, it is called peripheral T-cell lymphoma not otherwise specified (PTCL-NOS).
Is PTCL a rare disease?
PTCL is a group of rare, aggressive lymphomas. PTCLs are a subgroup of non-Hodgkin lymphoma (NHL).
How is PTCL diagnosed?
The diagnosis of PTCL-NOS is made based on the presence of typical histopathological features of lymphoma, an aberrant T-cell immunophenotype, often with a loss of CD5 and CD7, and a clonal T-cell receptor (TCR) gene rearrangement, in the appropriate clinical context.
How common is PTCL?
Based on the United States Surveillance, Epidemiology, and End Results (SEER) Registry, the incidence of PTCL is < 1 case per 100,000 people in the United States. In Europe and North America, T-cell and NK-cell lymphoma account for 5-10% of all cases of NHL whilst in Asia, this percentage is as high as 24%.
What is indolent lymphoma?
Indolent lymphoma is a type of non-Hodgkin’s lymphoma (NHL) that grows and spreads slowly. An indolent lymphoma usually won’t have any symptoms. Lymphoma is a type of cancer of the white blood cells of the lymphatic, or immune, system.
Are there any clinical trials focusing on PTCL?
Studies that focus specifically on PTCL are emerging, with the ultimate goal of improved understanding of disease biology and the development of more effective therapies. However, one of the difficulties in classifying and studying treatment options in clinical trials is the rarity of these subtypes.
Where does PTCL-NOS occur outside of the lymph nodes?
Although most patients with PTCL-NOS are diagnosed with their disease confined to the lymph nodes, sites outside the lymph nodes, such as the liver, bone marrow, gastrointestinal tract, and skin, may also be involved.
What is indolent peripheral T cell lymphoma not otherwise specified?
Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) is a heterogeneous group of mature T cell lymphomas which do not correspond to any specific subtype of mature T-cell lymphoma in current classifications. Some researchers have suggested that PTCL with low Ki-67 labeling index should be classified as indolent PTCL
How are collaborative groups help patients with PTCL?
Collaborative groups will aid in future efforts to find the best treatment strategies to improve the outcome for patients with PTCL.