Peripheral T-cell lymphoma

Peripheral T-cell lymphoma
Synonyms	PTCL
Pronounce	N/A
Specialty	N/A
Symptoms	Lymphadenopathy, fever, weight loss, night sweats
Complications	Infection, organ failure
Onset	Typically in adulthood
Duration	Chronic
Types	Angioimmunoblastic T-cell lymphoma, Anaplastic large cell lymphoma, Extranodal NK/T-cell lymphoma
Causes	Unknown, potential genetic and environmental factors
Risks	Age, gender, ethnicity, family history
Diagnosis	Biopsy, immunophenotyping, imaging studies
Differential diagnosis	B-cell lymphoma, Hodgkin lymphoma, reactive lymphoid hyperplasia
Prevention	None known
Treatment	Chemotherapy, radiation therapy, stem cell transplant
Medication	N/A
Prognosis	Variable, generally poor
Frequency	Rare
Deaths	N/A

(peh-RIH-feh-rul ... lim-FOH-muh) One of a group of aggressive (fast-growing) non-Hodgkin lymphomas that begin in mature T lymphocytes (T cells that have matured in the thymus gland and gone to other lymphatic sites in the body, including lymph nodes, bone marrow, and spleen). Also called mature T-cell lymphoma. Peripheral T-cell lymphoma refers to a group of T-cell lymphomas that develop away from the thymus. Examples include:

• Cutaneous T-cell lymphomas
• Angioimmunoblastic T-cell lymphoma
• Extranodal natural killer/T-cell lymphoma, nasal type
• Enteropathy type T-cell lymphoma
• Subcutaneous panniculitis-like T-cell lymphoma
• Anaplastic large cell lymphoma
• Peripheral T-cell lymphoma-Not-Otherwise-Specified

Treatment The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Brentuximub vedotin (Brand name: Adcetris) brentuximab vedotin (Adcetris) was approved for the treatment of adult patients with previously untreated systemic anaplastic large cell lymphoma (sALCL) or other CD30-expressing peripheral T-cell lymphomas (PTCL), including angioimmunoblastic T-cell lymphoma and PTCL not otherwise specified, in combination with cyclophosphamide, doxorubicin, and prednisone. In November 2016, it was approved for treatment of adult patients with primary cutaneous anaplastic large cell lymphoma. In August 2011, it was approved for the treatment of patients with systemic anaplastic large cell lymphoma (sALCL) after failure of at least one prior multi-agent chemotherapy regimen.
• Belinostat (Brand name: Beleodaq)belinostat (Beleodaq) was approved for the treatment of patients with relapsed or refractory peripheral T-cell lymphoma(PTCL).
• Pralatrexate (Brand name: Folotyn)pralatrexate (Folotyn) was approved for the treatment of patients with relapsed or refractory peripheral T-cell lymphoma.

NIH genetic and rare disease info

• NIH info on Peripheral T-cell lymphoma

Peripheral T-cell lymphoma is a rare disease.