Subcutaneous T-cell lymphoma (also known as a "panniculitis-like T-cell lymphoma") is a cutaneous condition that most commonly presents in young adults, and is characterized by subcutaneous nodules. Common symptoms include fever, fatigue, and pancytopenia.

Diagnosis

Classification

Subcutaneous panniculitis-like T-cell lymphoma, is a subtype of

Peripheral T-cell lymphoma Peripheral T-cell lymphoma refers to a group of T-cell lymphomas that develop away from the thymus or bone marrow. Examples include: * Cutaneous T-cell lymphomas * Angioimmunoblastic T-cell lymphoma * Extranodal natural killer/T-cell lymphoma, ...

. Peripheral T-cell lymphoma (PTCL) is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called

T-cells A T cell is a type of lymphocyte. T cells are one of the important white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell re ...

and natural killer cells (

NK cells Natural killer cells, also known as NK cells or large granular lymphocytes (LGL), are a type of cytotoxic lymphocyte critical to the innate immune system that belong to the rapidly expanding family of known innate lymphoid cells (ILC) and repres ...

) (see figure for an overview of PTCL subtypes). PTCL is a type of non-Hodgkin’s lymphoma (NHL). NHL affects two particular types of white blood cells:

B-cells B cells, also known as B lymphocytes, are a type of white blood cell of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or ...

and T-cells. PTCL specifically affects T-cells, and results when T-cells develop and grow abnormally. Subcutaneous Panniculitis-like T-cell Lymphoma is a rare type of lymphoma that infiltrates the subcutaneous fat but does not involve the skin. There are two subtypes – alpha-beta and gamma-delta. Patients with the gamma-delta subtype have a more aggressive clinical course. It is described as CD3+/ CD4-/ CD8+, with

CD30 CD30, also known as TNFRSF8 ( TNF receptor superfamily member 8), is a cell membrane protein of the tumor necrosis factor receptor family and a tumor marker. Function This receptor is expressed by activated, but not by resting, T and B cel ...

and CD56 usually negative.

Treatment

Historically, Subcutaneous Panniculitis-like T-cell Lymphoma, as with other types of T-cell lymphoma, was treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no drugs approved by the U.S. Food and Drug Administration (FDA) specifically for PTCL. There are two drugs, Pralatrexate and Romidepsin, which are both approved by the FDA for relapsed or refractory T cell lymphoma.

Anthracycline Anthracyclines are a class of drugs used in cancer chemotherapy that are extracted from ''Streptomyces'' bacterium. These compounds are used to treat many cancers, including leukemias, lymphomas, breast, stomach, uterine, ovarian, bladder ...

-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive an autologous or allogeneic

stem cell transplant Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood in order to replicate inside of a patient and to produce ...

. Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation. CHOP or CHOP-like regimens have been used.

Research

Pralatrexate is one compound currently under investigation to add to the frontline treatment of PTCL. It is currently approved by the FDA for relapsed or refractory T cell lymphoma. Side effects include mouth sores, which can be severe.

References

External links