what is treatment drug for peripheral t-cell lymphoma

by Chloe Streich Published 3 years ago Updated 2 years ago

Treatments currently being investigated singly or in combination include:

Alisertib (MLN8237)
Bendamustine (Treanda)
Bortezomib (Velcade)
Brentuximab vedotin (Adcetris) (approved for relapsed or refractory ALCL only)
Crizotinib (Xalkori)
GDP (gemcitabine, dexamethasone, and cisplatin)
Lenalidomide (Revlimid)
Nivolumab (Opdivo)
Panobinostat (Farydak)
Pembrolizumab (Keytruda)

For most subtypes of peripheral T-cell lymphoma (PTCL), the initial treatment is typically a combination chemotherapy regimen, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), CHOEP (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone), or other multidrug regimens.

Full Answer

What is the survival rate for T cell lymphoma?

Many new drugs are being studied in clinical trials for the treatment of PTCL, including: Alisertib Bendamustine (Treanda) Bortezomib (Velcade) GDP (gemcitabine, dexamethasone, and cisplatin) Lenalidomide (Revlimid) Nivolumab (Opdivo) Panobinostat (Farydak) Pembrolizumab (Keytruda) PI3K inhibitors

What are the stages of T cell lymphoma?

Treatments currently being investigated singly or in combination include: Alisertib (MLN8237) Bendamustine (Treanda) Bortezomib (Velcade) Brentuximab vedotin (Adcetris) (approved for relapsed or refractory ALCL only) Crizotinib (Xalkori) GDP (gemcitabine, dexamethasone, and cisplatin) Lenalidomide ...

What is the prognosis for T cell lymphoma?

Peripheral T-cell lymphoma, unspecified These lymphomas are generally treated the same way as diffuse large B-cell lymphoma (DLBCL). Chemo with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other drug combinations is used. For early-stage disease, radiation therapy may be added.

What is used in treatment of T-cell lymphoma?

Intensive chemo using several drugs is usually the main treatment. Often CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) is the chemo used. If the lymphoma is only in one area, radiation therapy may be used as well.Aug 3, 2021

Is there a cure for peripheral T-cell lymphoma?

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 is the survival rate of peripheral T-cell lymphoma?

The prognosis of patients with PTCL‐NOS is poor, and optimal therapy remains challenging. With standard anthracycline‐based therapy, the complete response rate ranges from 40% to 60%, with overall survival (OS) of 30–40% (Gallamini et al, 2004; Weisenburger et al, 2011).Apr 19, 2018

What medications are used to treat lymphoma?

BEACOPP includes the following drugs:

bleomycin (Blenoxane)
etoposide (Etopophos, Toposar, VePesid, VP-16)
doxorubicin (Adriamycin)
cyclophosphamide (Cytoxan)
vincristine (Oncovin)
procarbazine (Matulane)
prednisone (Rayos, Prednisone Intensol)

How long can you live with T-cell lymphoma?

Among all histological type subgroups, the prognosis of NK/T-cell lymphoma was the worst with the 3-year survival rate of only 25%u the 3-year survival rate was 40% in unspecified peripheral T-cell lymphoma group,and 85% in angioimmunoblast T-cell lymphoma group.

Is peripheral T-cell lymphoma painful?

Symptoms of peripheral T-cell lymphoma (PTCL)

The most common first sign of peripheral T-cell lymphoma (PTCL) is a painless lump. The lump can grow in the neck, armpit, or groin. The swelling is caused by the lymph nodes due to the presence of the lymphoma. For some patients, this swelling may be uncomfortable.

Which is worse B-cell or T-cell lymphoma?

Peripheral T-cell lymphomas have a worse prognosis than B-cell lymphomas: a prospective study of 361 immunophenotyped patients treated with the LNH-84 regimen.

What are the stages of T-cell lymphoma?

The staging of the most common type of CTCL, mycosis fungoides/Sézary syndrome (MF/SS) is classified into stages IA through IVB using the T (tumor, which for CTCL is patches or plaques), N (lymph node), M (presence of metastasis), B (presence of blood involvement) (TNMB) system.

Is peripheral T-cell lymphoma hereditary?

Sequencing studies have identified recurrent genetic alterations in nearly every PTCL subtype.Apr 1, 2018

What is the newly approved drug to treat lymphoma?

Today, the U.S. Food and Drug Administration approved Breyanzi (lisocabtagene maraleucel), a cell-based gene therapy to treat adult patients with certain types of large B-cell lymphoma who have not responded to, or who have relapsed after, at least two other types of systemic treatment.Feb 5, 2021

What is the latest treatment for lymphoma?

A drug called ibrutinib (Imbruvica) has been developed to shut down that pathway. It is being used and tested in a number of ways: In the last several years, the drug has been approved for the treatment of small lymphocytic lymphoma and Waldenstrom macroglobulinemia, both indolent non-Hodgkin lymphomas.Dec 1, 2021

Can Rituxan cure lymphoma?

RITUXAN is a prescription medicine used to treat adults with: Non-Hodgkin's Lymphoma (NHL): alone or with other chemotherapy medicines. Chronic Lymphocytic Leukemia (CLL): with the chemotherapy medicines fludarabine and cyclophosphamide.

Adult T-Cell Leukemia/Lymphoma

This lymphoma is linked to infection with the HTLV-1 virus. There are 4 subtypes, and treatment depends on which subtype you have. 1. The smolderin...

Angioimmunoblastic T-Cell Lymphoma

This fast-growing lymphoma might be treated first with steroids (such as prednisone or dexamethasone) alone, especially in older patients who might...

Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

This rare lymphoma is often confined to the nasal passages. Patients with stage I or II disease who are not fit for chemotherapy may be treated wit...

Enteropathy-Associated T-Cell Lymphoma

This lymphoma generally develops in the small intestine or colon. Intensive chemo using several drugs is usually the main treatment. Often CHOP (cy...

Anaplastic Large Cell Lymphoma (ALCL)

This fast-growing lymphoma mainly affects lymph nodes and is treated with chemo regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine,...

Peripheral T-Cell Lymphoma, Unspecified

These lymphomas are generally treated the same way as diffuse large B-cell lymphoma (DLBCL). Chemo with CHOP (cyclophosphamide, doxorubicin, vincri...

What are the treatments for lymphoma?

Standard lymphoma therapies may include chemotherapy, immunotherapy (like antibody drug conjugates) radiation, stem cell transplantation, and surgery. Patients diagnosed with rare forms of lymphoma should consult their medical team to find new promising therapies or to enroll into clinical trials. Treatments aimed at the skin, such as topical ...

What is the procedure called for CTCL?

In addition, a procedure called extracorporeal photopheresis (ECPP) is approved to treat people with CTCL. For this procedure, blood is removed from the patient and treated with ultraviolet light, and with drugs that become active when exposed to ultraviolet light.

What type of lymphoma starts with B cells?

Non-Hodgkin lymphoma (NHL) is generally divided into main 2 types, based on whether it starts in B lymphocytes (B cells) or T lymphocytes (T cells). There are many different types of T-cell lymphomas, and treatment can vary based on which type you have.

Can chemo cure lymphoma?

For more details on treatment, see Treating Acute Lymphocytic Leukemia (Adults) and Treating Childhood Leukemia. Although this lymphoma is fast-growing, if it hasn’t spread to the bone marrow when it’s first diagnosed, the chance of cure with chemo is quite good.

What is the treatment for lymphoma?

If the lymphoma is affecting the skin, it may be treated with radiation. Another option is chemo, using the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other combinations. The acute subtype also can be treated with either anti-viral drugs or chemo (typically the CHOP regimen).

Can lymphoma be treated with stem cell transplant?

If it responds well to treatment, a stem cell transplant might be considered. Anti-viral therapy is not helpful for the lymphoma subtype, so it is typically treated with chemotherapy. It can also involve the tissues around the brain and spinal cord, so chemo is given into the spinal fluid (intrathecal chemo) as well.

Can lymphoma be treated with chemotherapy?

Anti-viral therapy is not helpful for the lymphoma subtype, so it is typically treated with chemotherapy. It can also involve the tissues around the brain and spinal cord, so chemo is given into the spinal fluid (intrathecal chemo) as well. Treatment after chemo may include a stem cell transplant.

Can T-cell lymphoma be treated with radiation?

This rare lymphoma is often confined to the nasal passages. Patients with stage I or II disease who aren't healthy enough for chemotherapy may be treated with radiation therapy alone.

Can radiation therapy be used for lymphoma?

If the lymphoma is only in one area, radiation therapy may be used as well. But if these treatments work, a hole (perforation) can develop in the intestines (as the lymphoma cells die), so surgery might be done first to remove the part of the intestines containing the lymphoma.

Stage II

II: The cancer is in two or more lymph nodes in the region of the diaphragm (above or below).

Stage III

The cancer is in the lymph node areas on both sides of the diaphragm (above and below).

Stage IV

The cancer has spread throughout the body outside the lymph system, commonly in the liver, lungs, or bone marrow.

Drug combinations

While PTCL has no standard of care, most subtypes receive an initial treatment of a combination chemotherapy regimen.

Stem cell transplants

Doctors may recommend that fit individuals who responded well to initial intensive chemotherapy treatments undergo a stem cell transplant, which studies suggest may improve outcomes and lead to longer remissions.

Other therapies

Doctors may give antiviral drugs to people with PTCL caused by viruses in addition to a chemotherapy regimen. Those with localized PTCL, such as extranodal NK/T-cell lymphoma and angioimmunoblastic T-cell lymphoma, may benefit from radiation therapy. Some may also benefit from chemotherapy with radiation (chemoradiation).

Clinical trials

Since these standard treatment options are only based on small trials and lack randomized trials, response to these treatments is usually unsatisfactory. Doctors may recommend that people participate in clinical trials that involve new drugs or drug combinations.

What is a peripheral T lymphoma?

Peripheral T-cell lymphomas (PTCLs) are a rare and diverse group of neoplasms with a poor prognosis. Management of these disorders has been largely extrapolated from the treatment of aggressive B-cell lymphomas; however, therapeutic responses to this approach are neither adequate nor durable for most patients with PTCL. Given the rarity of PTCL, much of the literature consists of studies with small sample size and anecdotal case reports. Therefore, no consensus exists on the best therapeutic strategy for either newly diagnosed or relapsed/refractory PTCL. This article reviews promising novel approaches in the treatment of PTCL and its subtypes. Investigation into the pathogenesis of PTCL has also identified new targets for treatment. These emerging therapies include new uses of existing agents and the development of novel agents specifically targeted against T-cell lymphoma. Results using antimetabolites, immunotherapies, and histone deacetylase inhibitors have been particularly encouraging. These novel therapies are being tested as single agents and in combination with conventional lymphoma regimens in the frontline and salvage settings. Because of the rarity and heterogeneity of PTCL, national and international cooperation is needed to conduct the clinical studies required for the development of more effective treatment paradigms. These efforts are ongoing and will hopefully guide new strategies to improve the historically poor outcome of PTCL.

What is a T cell lymphoma?

Peripheral T-cell lymphomas (PTCLs) are a rare and heterogeneous group of disorders with a poor prognosis whose optimal treatment is uncertain. The World Health Organization classification recognizes 13 different types of mature T-cell neoplasms, grouped into leukemic, extranodal, and nodal types. [1] Of these, cutaneous T-cell lymphoma (CTCL) and T-cell leukemias are clinically unique entities and will not be discussed in this article except as they relate to the development of therapies for PTCL. In North America, PTCL-unspecified (PTCL-U), systemic anaplastic large-cell lymphoma (ALCL), and angioimmunoblastic T-cell lymphoma (AITL) are the most common forms of PTCL, whereas nasal-type extranodal natural killer/T-cell lymphoma (nNK/T) is more frequent in the Far East. [2] Except for patients with ALCL that expresses anaplastic lymphoma kinase (ALK), those with PTCL have a poor prognosis compared with patients with aggressive B-cell non-Hodgkin’s lymphoma (NHL), with a 5-year disease-free survival of less than 30% in large series. [2,3] The International Prognostic Index for aggressive NHL, the most frequently used clinical prognostic score, stratifies patients with PTCL. [4,5] However, patients with PTCL are more likely to present with high-risk features, such as advanced stage and extranodal sites, and have a markedly worse survival compared with those who have aggressive B-cell NHL of similar risk. Another prognostic index specifically for PTCL-U (PIT) is based on age, performance status, lactate dehydrogenase, and bone marrow involvement. [6] Modifying the PIT to incorporate proliferation seems to further stratify survival compared with the International Prognostic Index or unmodified PIT. [7]

Is chop a good treatment for PTCL?

Although CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) is the most commonly used initial therapy, this approach is extrapolated from the treatment of aggressive B-cell NHL and is not adequate for PTCL , because most patients have refractory disease or experience relapse.

Symptoms of T-cell lymphoma

Symptoms of T-cell lymphoma vary widely based on the type of lymphoma, but below are some of the more common ones, including:

Peripheral T-cell lymphomas

Cutaneous T-cell lymphoma begins in the skin and makes up about 5 percent of all lymphomas, according to the ACS.

What is PTCL lymphoma?

PTCL is classified as a subtype of non-Hodgkin’s lymphoma (NHL). NHL affects two particular types of white blood cells: B-cells and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally. It is PTCL’s origin in the lymphatic system that gave it the name peripheral T-cell lymphoma.

What is the most common type of lymphoma?

The most common subtype is called PTCL-not-otherwise specified (PTCL-NOS) and is most frequently diagnosed in individuals living in North America and Europe. Anaplastic large cell lymphoma (ALCL) is common in North America and Europe, whereas angioimmunoblastic T-cell lymphoma (AITL), the second most common subtype, is found more often in Europe.

Where does PTCL come from?

It is PTCL’s origin in the lymphatic system that gave it the name peripheral T-cell lymphoma. In the case of PTCL, the term “peripheral” does not refer to the extremities, but identifies PTCL as a cancer that arises in the lymphoid tissues outside of the bone marrow such as lymph nodes, spleen, gastrointestinal tract, and skin.