T-cell lymphoma

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T-cell lymphoma
stem cell transplant

T-cell lymphoma is a rare form of cancerous

cancerous.[2]

T-cell lymphoma is categorized under

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.

radiotherapy.[2] The effectiveness of these treatments is often varied between subtypes with most receiving a poor outcome with high relapse rates.[4]

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.[4] Most classifications are basic with many still under the title of ‘provisional categories’ in the World Health Organization Classification of disease.[5]

Common

Rare

Symptoms and signs

Symptoms of Mycosis fungoide

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.[4]

The hemophagocytic syndrome (HPS)

lymph nodes, these can include; Extranodal NK/T-cell lymphoma, nasal type, Cutaneous T-cell lymphoma (CTCL), etc.[5]

Swollen lymph nodes

T-cell lymphoma which develops from the lymph nodes commonly causes symptoms as such swollen lymph nodes.[6] 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.[citation needed]

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.[4]

Cause

Although there is no definitive cause for most T-cell lymphoma subtypes, a series of risk factors have been linked and associated with the increased likelihood of contracting the disease.

Risk factors

Family history: A family history of

malignancies has been linked to an increased association with most T-cell lymphoma subtypes. This link is especially elevated among individuals 50 years or younger.[2]
However, the link is still considered as a hypothesised risk meaning that research conducted on this link have been insufficient or inconclusive.

Autoimmune conditions: Autoimmune conditions are commonly considered as a risk factor that has been associated with non-Hodgkin lymphomas, with

Organ transplants and immunosuppressant:

immunosuppressant therapy is considered an established risk factor for all Non-Hodgkin lymphoma, including T-cell lymphoma. This risk factor elevates the risk of contracting T-cell lymphoma.[2]

Infectious Agents: Several infectious agents have been linked to a higher risk of T-cell lymphoma by providing a compromised immune function allowing the establishment of lymphomas. Of these

Human T-cell leukemia virus-1 (HTLV1) are considered established risks.[7]

Epstein–Barr virus is a largely common virus with more than 90% of individuals exposed to the virus in their lifetime. EBV has been consistently associated with many lymphoproliferation disorders, of these EBV-associated T-cell lymphomas include Epstein–Barr virus–associated lymphoproliferative diseases, angioimmunoblastic T-cell lymphoma (AITL), extranodal NK/T-cell lymphoma, nasal type, and Peripheral T-cell lymphoma not otherwise specified (PTCL, NOS).[8]

The

human T-cell leukemia virus-1 is endemic in regions such as Japan and the Caribbean and has been associated with the increased risk of T-cell lymphoma such as Adult T-cell leukemia/lymphoma (ATL).[9] HTLV-1 has been attributed to 56% and 78% of all ATL cases in Japan and the Caribbean respectively.[8]

Diagnosis

Biopsy Procedure

The diagnosis of T-cell lymphoma varies largely between the subtypes. Some subtypes like anaplastic large-cell lymphoma have an exceptional diagnostic rate however,

X-rays may also be used for diagnostic purposes.[1]

Treatment

Stem cell Transplant

Treatment for T-cell lymphoma varies widely due to the large variability in the subtypes. Due to the lack of research performed in understanding the nature of T-cell lymphoma pathogenesis, a majority of cases will often have poor outcomes for the treatment or will relapse.[3] However, new research into new therapy methods have been made to help reduce the mortality rates and risk of relapse.[10][2]

Chemotherapy

stem cell transplants.[2]

Radiotherapy

electron beams in radiotherapy only penetrate to the level of the dermis, it is a common method of treatment for skin lymphoma which may only occur locally such as Cutaneous T-cell lymphoma, however, it is not recommended for patients with systemic lymphoma conditions.[6]

Stem cell transplant

Stem Cell Transplants are a common method of treatment which can either be used in conjunction with chemotherapy to improve remission and effectiveness or it can be used with relapsed lymphoma patients.

allogeneic stem cell transplant (alloHCT) in which a related or unrelated healthy donor will donate their stem cells to the patient.[3] Stem cells are collected from the bone marrow and are a type of cell capable of self-renewal and can differentiate into all types of cells,[2] this can be utilised for patients with T-cell lymphoma and has seen effective results in treating some subtypes, especially Angioimmunoblastic T-cell lymphoma.[8]

Allogenic stem cell transplants are mainly used when the patient lacks adequate healthy stem cells for an autologous stem cell transplant or has relapsed after prior autologous stem cell transplant treatments.

myeloablative treatment is given alongside stem cell transplants to reduce the immune response.[2]

Monoclonal antibodies

Monoclonal antibodies (mAb) utilizes

tumours, it induces apoptosis of the tumour through the obstruction of survival pathways.[11] Monoclonal antibodies can be used as a single treatment agent, however, are more effective when used concurrently with chemotherapy to improve survival and remission.[2] Most commonly used monoclonal antibody used to treat T cell lymphoma include; alemtuzumab and denileukin difititox.[3]

Nucleoside analogs

immunosuppressive abilities and acts by inhibiting viral replication and prevent the spread of the cancerous growth.[3]
Nucleoside analogs are one of the most active class of drug used to treat T-cell lymphoma.

Other

Other non-tradition or new treatment options include:

HDAC inhibitors
.

Epidemiology

While the incidence of Non-Hodgkin's lymphoma has plateaued, the rate of T-cell lymphomas has gradually increased over the past few years. However, due to the low frequency and lack of research performed on the disease, the number of cases is relatively underrepresented compared to other non-Hodgkin lymphomas.[3]

Cases are more common in those of Native American descent followed by Caucasian ancestry,[2] however, the epidemiology can vary greatly between the different subtypes. The incidences[spelling?] of T-cell lymphoma are slightly higher in men than in women in all categories of race[6] with cases increasing in frequency with age for most subtypes.[2]

In Asia, T/NK-cell

human T-cell leukemia virus-1 (HTLV1) and Epstein–Barr virus (EBV). While enteropathy-associated T-cell lymphoma (EATCL) is more common among Irish and Welsh populations.[2]

See also

References

  1. ^ a b c d Quesenberry, Peter J.; Castillo, Jorge J. (2013). Non-Hodgkin Lymphoma Prognostic Factors and Targets. NY: Humana Press.
  2. ^ a b c d e f g h i j k l m n o p q Foss, Francine (2013). T-cell Lymphomas. Totowa, NJ: Humana Press.
  3. ^ a b c d e f g Querfeld, Christiane; Zain, Jasmine; Rosen, Steven T (2019). T-Cell and NK-Cell Lymphomas From Biology to Novel Therapies. Cham: Springer International Publishing.
  4. ^
    PMID 26250731
    .
  5. ^ a b World Health Organization. "International Classification of Diseases 11th Revision". World Health Organization. Retrieved 5 October 2020.
  6. ^ .
  7. .
  8. ^ a b c d e Evens, Andrew M.; Blum, Kristie A. (2015). Non-Hodgkin Lymphoma Pathology, Imaging, and Current Therapy. Cham: Springer International Publishing.
  9. .
  10. ^ .
  11. .