T-cell lymphoma refers to a group of aggressive blood cancers that originate from mature or immature T cells. As T cells play a key role in the immune system, their malignancy can weaken the body's ability to fight infections and other diseases. In this article, we delve deeper into the types, causes, symptoms, diagnosis and treatment of this rare but serious type of non-Hodgkin lymphoma (NHL).
Types of T-cell Lymphoma
There are several subtypes of T-cell lymphoma categorized based on the stage of development and function of the malignant T cells. Some of the main types include:
Peripheral T-cell Lymphoma
Peripheral T-cell Lymphoma (PTCL) is an umbrella term for several subtypes that arise from mature, functional T cells in lymphoid tissues outside the bone marrow. The most common PTCL subtypes are:
- Angioimmunoblastic T-cell Lymphoma
- Anaplastic Large Cell Lymphoma
- Others like Adult T-cell Leukemia/Lymphoma caused by HTLV-1 virus.
Cutaneous T-cell Lymphoma
Cutaneous T-cell Lymphoma (CTCL) involves the skin and can present with rashes, lesions or thickened patches of skin. Mycosis fungoides and Sézary syndrome are the most prevalent CTCL subtypes.
Primary T-cell Lymphoma of the Central Nervous System
This rare type of T-cell lymphoma starts in the brain, spinal cord or meninges. Early symptoms include headaches, confusion and seizures.
Causes and Risk Factors
Unlike B-cell lymphomas, the causes of most T-cell lymphomas remain unknown. However, certain risk factors have been linked to increased odds of developing the disease:
- Advanced age: Risk rises after 50 years.
- Weakened immune system: Due organ transplant, HIV/AIDS or autoimmune conditions.
- Family history of lymphoma or related cancers.
- Viral infections: HTLV-1 causes ATLL; EBV linked to some subtypes.
Genetic defects that impair T-cell development and function have also been tied to higher predisposition. Exposure to ionizing radiation or certain chemicals may be additional contributory factors.
Symptoms and Diagnosis
Due to the diverse nature of T-cell lymphomas, symptoms depend on the specific type and site of involvement. Common initial signs may include:
- Enlarged lymph nodes in neck, underarm or groin
- Fever and weight loss
- Fatigue and night sweats
- Skin lesions or rashes for CTCL patients
Diagnosis requires a combination of physical exam, blood tests, imaging scans and tissue biopsies. Procedures may involve lymph node biopsy, bone marrow aspiration or skin/tissue biopsies for histopathology and immunophenotyping. Molecular and genetic testing helps classify and stage the disease accurately.
Staging and Prognosis
Once diagnosed, T-Cell Lymphoma is staged according to the Ann Arbor staging system. This gives an idea about prognosis and guides treatment planning. Common risk factors impacting outlook include:
- Advanced stage at diagnosis
- High lactate dehydrogenase levels
- Elevated soluble interleukin-2 receptor levels
- B symptoms like fever and weight loss
- Performance status and age of patient
- Subtype - some have worse survival than others
Overall, the 5-year survival for T-cell lymphoma patients ranges from 30-70% depending on individual risk factors and how aggressively the cancer responds to initial therapies. Early detection and treatment improves survival chances significantly.
Treatment Approaches
A multidisciplinary care team designs individualized treatment regimens based on disease factors. Primary treatment goals are long-term remission and cure. Common therapeutic options include:
Chemotherapy
Combination chemotherapy regimens containing drugs like CHOP, EPOCH orHyperCVAD are frequently employed as first-line treatment. Allogeneic stem cell transplant may be recommended after chemotherapy for relapsed/refractory cases.
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