Types of Peripheral T-Cell Lymphoma

Question 1

CHOP-based chemotherapy

Answer 1

cyclophosphamide, doxorubicin, vincristine and

prednisone

Question 2

EPOCH

Answer 2

etoposide, vincristine, doxorubicin,

cyclophosphamide and prednisone

Question 3

Relapsed PTCL patients are usually treated with ICE followed by an autologous stem cell transplant

Answer 3

ifosfamide, carboplatin and etoposide

Question 4

CD30

Answer 4

CD30 is a membrane protein detectable by immunohistochemistry (IHC)

Question 5

Brentuximab vedotin (BV)

Answer 5

is a CD30-directed immunoconjugate

Also in 2011, the FDA approved brentuximab vedotin (Adcetris) for the treatment of relapsed or refractory systemic ALCL. Brentuximab vedotin in combination with other treatments, including CHOP and bendamustine (Treanda), are currently underway, and the drug is also being tested in other PTCL types.

Question 6

Peripheral T-cell lymphoma, NOS (PTCL, NOS)

Answer 6

Peripheral T cell lymphoma, not otherwise specified (PTCL, NOS) is a heterogeneous group of predominantly nodal T cell lymphomas derived from various types of mature T cells that do not meet the criteria for the other specifically defined subtypes of PTCL. PTCL, NOS accounts for the largest number of patients with PTCL in Western countries, accounting for 30% of the PTCL and 4% of NHLs overall.

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. This group of PTCLs is aggressive and requires combination chemotherapy upon diagnosis.

Question 7

Angioimmunoblastic T cell lymphoma (ATCL)

Answer 7

This type of lymphoma often responds to milder therapies, such as steroids, although it often progresses and requires chemotherapy and other medications. In advanced cases, bone marrow transplantation may be used.

Question 8

Anaplastic large cell lymphoma (ALCL)

Answer 8

· Anaplastic large cell lymphoma (ALCL):About 2% of lymphomas are of this type. It is more common in young people (including children), but it can also affect older adults. This type of lymphoma tends to be fast-growing, but many people with this lymphoma can be cured.
· There are different forms of ALCL:
· Primary cutaneous ALCLonly affects the skin
· Systemic ALCLcan affect the lymph nodes and other organs, including the skin. Systemic ALCL is divided into 2 types based on whether the lymphoma cells have a change in the ALK gene. ALK-positive ALCL is more common in younger people and tends to have a better prognosis (outlook) than the ALK-negative type.

Question 9

Hepatosplenic gamma/delta T cell lymphoma

Answer 9

Hepatosplenic Gamma-Delta T-Cell Lymphomais an extremely rare and aggressive disease that starts in the liver or spleen.

Question 10

Extranodal T/NK-cell lymphoma, nasal type

Answer 10

Nasal NK/T-Cell Lymphoma involves natural killer (NK) cells, which are closely related to and often have features that overlap with T-cells. Although this aggressive lymphoma is very rare in the United States, it is more common in Asia and parts of Latin America, leading researchers to suspect that some ethnic groups may be more prone to this cancer. This type of lymphoma is associated with the Epstein-Barr virus and often involves the nasal area, trachea, gastrointestinal tract, or skin.

Question 11

Enteropathy-type intestinal T-cell lymphoma

Answer 11

Enteropathy-type T-cell Lymphoma is an extremely rare subtype that appears in the intestines and is strongly associated with celiac disease.

Question 12

Frontline treatment regimen for Peripheral T-Cell Lymphoma

Answer 12

For most subtypes of PTCL, the frontline treatment regimen is typically a combination chemotherapy, such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, prednisone), or other multi-drug regimen. Because most patients with PTCL will relapse, some oncologists recommend giving high-dose chemotherapy followed by an autologous (in which patients receive their own stem cells) stem cell transplant to some patients who had a good response to their initial chemotherapy.

Question 13

GND

Answer 13

vinorelbine (Navelbine) and doxorubicin (Doxil) in a regimen called GND

Gemcitabine appears effective against some forms of relapsed PTCL and is often given in combination with other chemotherapies, including vinorelbine (Navelbine) and doxorubicin (Doxil) in a regimen called GND

Question 14

DHAP

Answer 14

dexamethasone, cytarabine, cisplatin

Question 15

ESHAP

Answer 15

etoposide, methylprednisolone, cytarabine, and cisplatin

Question 16

Signs / symptoms of NHL

Answer 16

Enlarged lymph nodes
Fever
Sweating and chills
Weight loss
Fatigue (extreme tiredness)
Swollen abdomen (belly)
Feeling full after only a small amount of food
Chest pain or pressure
Shortness of breath or cough