T-cell lymphoma is a rare form of cancerous lymphoma affecting T-cells. Lymphoma arises mainly from the uncontrolled proliferation of lymphocytes, such as T-cells, and can become cancerous. T-cell lymphoma is categorized under Non-Hodgkin lymphoma (NHL) and represents less than 15% of all Non-Hodgkin's diseases in the category. T-cell lymphomas are often categorised based on their growth patterns as either aggressive (fast-growing) or indolent (slow-growing). Although the cause of T-cell lymphoma is not definitive, it has been associated with various risk factors and viruses such as Epstein–Barr virus (EBV) and human T-cell leukemia virus-1 (HTLV1). The prognosis and treatment of T-cell lymphoma can vary drastically based on the specific type of lymphoma and its growth patterns. Due to their rarity and high variability between the different subtypes, the prognosis of T-cell lymphoma is significantly worse than other Non-Hodgkin lymphoma. The treatment of T-cell lymphoma is often similar to other Non-Hodgkin lymphomas with early-stage treatments consisting of chemotherapy and/or radiotherapy. The effectiveness of these treatments is often varied between subtypes with most receiving a poor outcome with high relapse rates.
Types There are many types and variations of T-cell lymphoma, each with vastly different symptoms, survival, and prognosis. The classification of T-cell lymphoma has been difficult to accomplish due to the lack of understanding of their biology. Most classifications are basic with many still under the title of 'provisional categories' in the World Health Organization Classification of Diseases.
Common Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS): Most common type of Peripheral T-cell lymphoma (PTCL), comprising subtypes which cannot be classified as either nodal, extra-nodal, or leukemic Angioimmunoblastic T-cell lymphoma (AITL): Aggressive form of T-cell lymphoma. Anaplastic large cell lymphoma (ALCL): ALCL has four distinct types: ALK-positive anaplastic large cell lymphoma: an aggressive, systemic ALCL that strongly expresses anaplastic lymphoma kinase, i.e. ALK. ALK-negative anaplastic large cell lymphoma: an aggressive, systemic ALCL that does not express ALK. Primary cutaneous anaplastic large cell lymphoma: a less aggressive ALCL that commonly presents as skin tumors. Breast cancer-associated anaplastic large cell lymphoma: a less aggressive ALCL that occurs around and is caused by breast implants. Adult T-cell leukemia/lymphoma (ATL): Aggressive T-cell lymphoma, associated with RNA retrovirus, human T-cell leukemia virus type-1 (HTLV1) Extranodal NK/T-cell lymphoma, nasal type (ENKTL): Aggressive T-cell lymphoma, usually associated with Epstein–Barr virus (EBV) Cutaneous T-cell lymphoma (CTCL): can be indolent or aggressive Mycosis fungoides Sézary syndrome
Rare Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) Systemic Epstein–Barr virus-positive T-cell Lymphoproliferative Disorders of Childhood (EBVTCLD): A very aggressive group with association with Epstein–Barr virus (EBV) Primary intestinal T-cell lymphomas Enteropathy-associated T-cell lymphoma (EATL) Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). Hepatosplenic T-cell lymphoma (HSTCL)
Symptoms and signs
Differences in T-cell lymphoma subtypes extend to the clinical characteristics and symptoms of the disease with each varying drastically. As a result, there is almost no universally known symptom that can be applied to all T-cell lymphoma subtypes.
The hemophagocytic syndrome (HPS) Hemophagocytic syndrome has been associated with most T-cell lymphoma subtypes, and is commonly characterized by fevers, reduction of lymphocyte numbers, enlarged liver or spleen, and liver dysfunction. These symptoms are especially common in extranodal T-cell lymphoma subtypes which develop outside the lymph nodes, such as extranodal NK/T-cell lymphoma, nasal type, and cutaneous T-cell lymphoma (CTCL).
Swollen lymph nodes T-cell lymphoma which develops from the lymph nodes commonly causes symptoms such as swollen lymph nodes. The swelling normally will not cause any pain and can be felt or seen as lumps on the surface of the skin. Nodal T-cell lymphoma subtypes such as peripheral T-cell lymphoma will often develop this symptom.
Skin infections T-cell lymphoma can cause eczema or rash-like symptoms where small red patches will appear around the skin. These patches will often be irritated and may appear slightly lighter in colour compared to the rest of the skin. Occasionally, small lumps will develop which may rupture and cause the surface layer of the skin to break open. This is especially common in Cutaneous T-cell lymphoma subtypes.
Cause
There is no definitive cause for most T-cell lymphoma subtypes, but a variety of risk factors have been linked to an increased likelihood of developing the disease.
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