Clinical manifestation of T-cell lymphoma depends on the type of lymphoma and the organs involved. Most of the T-cell lymphomas are high-grade with aggressive signs and symptoms that may lead to death if left untreated. Few subtypes may show indolent clinical symptoms which may or may not become aggressive after a period of time.

• T-prolymphocytic leukemia – is a typical aggressive form of lymphoma presenting with splenomegaly and lymphadenopathy.  
• T-cell large granulocyte leukemia – the clinical manifestation of this subtype is indolent with cytopenia and neutropenia as the most common symptoms. Some patients may have splenomegaly and lymph node enlargement. 
• Aggressive NK cell leukemia – this lymphoma presents with acute systemic symptoms including fever, jaundice, lymphadenopathy, and hepatomegaly. The condition may lead to liver dysfunction and even multi-organ failure. 
• ATL – the main clinical presentations of this type include lymphadenopathy and hepatosplenomegaly. Patients may also show lesions in the central nervous system, skin, and bones. Many patients also present with B symptoms and increased calcium levels. 
• PTCL – this type is characterized by increased presence of B-symptoms. The clinical course of the condition is aggressive. 
• Angioimmunoblastic T cell lymphoma – systemic symptoms and generalized lymphadenopathy are the characteristic symptoms of this type. Many patients also present with hepatosplenomegaly and pruritus. Polyarthritis and ascites are also frequently seen. 
• ALCL – more than three-fourth of the patients show B symptoms. Skin, liver, lungs, bone marrow, bones and soft tissue may also be involved in the disease. 
• Nasal-type T-cell lymphoma – it affects nasal structures, with facial edema, nasal obstruction as common symptoms. They may present with bulky tumors which may be invasive. The cells may invade sinuses, oral cavity and orbit. 
• Enteropathy-associated T-cell lymphoma – often seen in elderly patients, diarrhea and abdominal pain are commonly seen. Bowel obstruction, perforation and ulceration may be seen in some. Lesions are often seen in jejunum and ileum. 
• Hepatosplenic T-cell lymphoma – typical condition involves liver, spleen and bone marrow resulting in cytopenia and thrombocytopenia [11]. Affected tissues will show sinusoidal infiltration of malignant cells. 
• Subcutaneous panniculitis-like T-cell lymphoma -- subcutaneous nodules and ulceration are the most common clinical manifestations of this subtype. As the disease progresses lymphadenopathy and systemic involvement is common. 

• Generalized Lymphadenopathy

  Aggressive clinical course with generalized lymphadenopathy or mass lesions. Localized lymphadenopathy, viral syndrome, viral serologies. [surgpathcriteria.stanford.edu]

  A 64-year-old woman presented with generalized lymphadenopathy and systemic manifestations. The examination of a biopsy specimen revealed peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) expressing cytotoxic molecules. [ncbi.nlm.nih.gov]

  Clinical presentation The clinical spectrum includes high fevers, generalized lymphadenopathy, a skin rash, and hepatosplenomegaly. [radiopaedia.org]

  Most patients present with generalized lymphadenopathy, hepatosplenomegaly, skin rash and general symptoms (fever, weight loss). Polyclonal hypergammaglobulinemia is a common finding. [atlasgeneticsoncology.org]

• Splenomegaly

  Splenomegaly and adenopathy in mesentery, retro peritoneum and lymph node chains in the limbs were observed. Bone marrow examination showed findings compatible with T-cell leukemia/lymphoma; and it was ELISA positive for HTLV-1/2. [ncbi.nlm.nih.gov]

  T-prolymphocytic leukemia – is a typical aggressive form of lymphoma presenting with splenomegaly and lymphadenopathy. [symptoma.com]

  Splenomegaly can also be seen in rare cases, particularly in a late stage of disease. [archivesofpathology.org]

  In addition, regression of splenomegaly and/or hepatomegaly is usually a gradual, slow process, and in many cases complete resolution is never achieved, even in the case of CR. [hematologyandoncology.net]

• Fever

  CASE PRESENTATION: Case one is a 19-year-old female presented with fever and liver dysfunction, and was diagnosed as EBV-associated chronic active hepatitis. [ncbi.nlm.nih.gov]

• Pain

  Case two is a 37-year-old male complaining bilateral blurred vision and recurrent abdominal pain. Panuveitis was diagnosed and anterior inflammation did not release after topical steroid. [ncbi.nlm.nih.gov]

  He had occasional bloody discharge, without pain, and did not have constitutional or other systemic symptoms. Multiple skin biopsies eventually confirmed cutaneous T-cell lymphoma. Computed tomography revealed no lymphadenopathy or metastases. [nejm.org]

  The liver and spleen might be enlarged, causing bloating or tummy (abdominal) pain. Lymphoma elsewhere in your gut might cause pain, sickness, diarrhoea or vomiting. The skin can be affected by itchy red patches. [lymphoma-action.org.uk]

• Weight Loss

  A 24-year-old male, presented with a 2-week history of fever, drenching night sweats and nonquantified weight loss. [ncbi.nlm.nih.gov]

  […] if extralymphatic site involved Stage IV Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement Systemic Symptoms in 6 months preceding admission Fever, night sweats, 10% weight [surgpathcriteria.stanford.edu]

  There are also other symptoms associated with the disease, including night sweats or fevers, unexplainable weight loss or lack of appetite, and in more severe cases, breathing problems, pain, distention and fatigue are also common. [lymphomanewstoday.com]

  You might have: tummy (abdominal) pain weight loss diarrhoea, which might have blood in it fatigue an itchy rash. You might be malnourished (not getting enough nutrients) if you are not absorbing food properly. [lymphoma-action.org.uk]

  She reported non-quantified weight loss but no fever or other general symptoms. The physical examination did not show adenomegalia, hepatosplenomegalia and other abnormalities. [scielo.br]

• Lymphadenopathy

  Aggressive clinical course with generalized lymphadenopathy or mass lesions. Localized lymphadenopathy, viral syndrome, viral serologies. [surgpathcriteria.stanford.edu]

  Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. [ncbi.nlm.nih.gov]

  […] with Dysproteinemia Angioimmunoblastic Lymphadenopathy with Dysproteinemia Tweets by @WebPathology Slide Index Brain Breast Head & Neck Mediastinum Peritoneum Genitourinary Lymph Node/Spleen Normal Histology Lymph Node Spleen Hematopathology Gynecologic [webpathology.com]

  Computed tomography revealed no lymphadenopathy or metastases. No evidence of bone marrow involvement was found. The patient received a course of radiotherapy, with a good response (Panel B). Three months later, he presented with ataxia. [nejm.org]

• Fatigue

  Ten days later while in rehabilitation, he started complaining of progressive fatigue and altered mental status was noted. He was found to have a calcium level of 15.5 and was admitted to the intensive care unit for management and further workup. [ncbi.nlm.nih.gov]

  Radiation may cause temporary skin irritation and fatigue. Extracorporeal photopheresis This is used to treat mycosis fungoides or Sézary syndrome. [healthline.com]

  In acute ATLL, symptoms develop rapidly and include fatigue, skin rash and enlarged lymph nodes in the neck, armpit and groin. [leukaemia.org.au]

• Dyspnea

  We describe a 55-year man who presented with cough, sputum, dyspnea on exertion, and a chest computed tomography scan shows diffuse ground glass opacities (GGOs), suggestive of an interstitial lung disease. [ncbi.nlm.nih.gov]

  Sign In A 53-year-old man presented with fever, dyspnea, and rapidly progressive skin lesions. A biopsy specimen obtained from a lesion showed extranodal natural killer T-cell lymphoma. Kuan-Yu Chi, M.D. [nejm.org]

  They consist of: Fever[19][20] Night sweats[19][20] Weight loss[19][20] Anemia, bleeding, increase susceptibility to infections[21] Other symptoms: Loss of appetite or anorexia[20] Fatigue[19][20] Respiratory distress or dyspnea[20] Itching[19][20] Mouth [en.wikipedia.org]

• Abdominal Pain

  Case two is a 37-year-old male complaining bilateral blurred vision and recurrent abdominal pain. Panuveitis was diagnosed and anterior inflammation did not release after topical steroid. [ncbi.nlm.nih.gov]

  Hepatosplenic T-cell lymphoma People with this extremely rare, aggressive disease, which starts in the liver or spleen, experience fever, abdominal pain, weakness and swelling of the liver and spleen. [seattlecca.org]

  The liver and spleen might be enlarged, causing bloating or tummy (abdominal) pain. Lymphoma elsewhere in your gut might cause pain, sickness, diarrhoea or vomiting. The skin can be affected by itchy red patches. [lymphoma-action.org.uk]

• Diarrhea

  Here, we report a case of Type II EATL, in which the patient with repeated diarrhea was diagnosed as ulcerative colitis and amebic infection at the beginning, but her symptom had no improvement after therapy. [ncbi.nlm.nih.gov]

  The most common side effects of Adcetris plus chemotherapy included nerve damage (peripheral neuropathy), nausea and vomiting, diarrhea, low white blood cell counts, fatigue, mouth sores, constipation, hair loss, fever and low red blood cell count (anemia [europeanpharmaceuticalreview.com]

  Side effects of chemotherapy can include: hair loss nausea and vomiting constipation or diarrhea anemia, a shortage of red blood cells, leading to fatigue, weakness, and shortness of breath neutropenia, a shortage of white blood cells, which can leave [healthline.com]

• Intestinal Perforation

  These people are often in a very weakened state and may have intestinal perforation (an abnormal hole in the wall of the intestine) when they are diagnosed.. [lymphoma.org.au]

  It most frequently involves the small intestine; however, it could involve the colon and stomach. The course is aggressive, and death is usually due to multifocal intestinal perforation caused by refractory malignant ulcers. [pubs.rsna.org]

• Early Satiety

  History of present illness was notable for a 6-month history of nausea, emesis, early satiety, and severe left upper quadrant abdominal pain that radiated to her shoulder. [hematologyandoncology.net]

• Palpitations

  CASE PRESENTATION: An 83-year-old Caucasian man presented to an emergency department with palpitations and was found to have atrial fibrillation. [ncbi.nlm.nih.gov]

• Hepatosplenomegaly

  Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. [ncbi.nlm.nih.gov]

• Hepatomegaly

  The remainder of the physical examination was unremarkable; no lymphadenopathy or hepatomegaly was noted. [hematologyandoncology.net]

  Hepatomegaly and splenomegaly are also very common. The majority of patients will have “B” symptoms including fevers, night sweats, and/or unintentional weight loss. [cancertherapyadvisor.com]

  Palabras clave: Insuficiencia hepática aguda Linfoma de células T Citocinas Enfermedad de células falciformes Hepatomegaly and alterations in hepatic function are common to all patients with sickle-cell disease. [analesdepediatria.org]

  Aggressive NK cell leukemia – this lymphoma presents with acute systemic symptoms including fever, jaundice, lymphadenopathy, and hepatomegaly. The condition may lead to liver dysfunction and even multi-organ failure. [symptoma.com]

• Jaundice

  Priest Jaundice as a paraneoplasic phenomenon in a T cell lymphoma Gastroenterology, 97 (1989), pp. 1319-1322 [12.] S. Holt, Z. Varghese, M. Jarmulowicz, R. Marley, P. Amlot, M. [analesdepediatria.org]

  Aggressive NK cell leukemia – this lymphoma presents with acute systemic symptoms including fever, jaundice, lymphadenopathy, and hepatomegaly. The condition may lead to liver dysfunction and even multi-organ failure. [symptoma.com]

  Hepatic involvement may be found in 31% of patients with the acute or lymphoma type and is not infrequently manifested by jaundice (30%) and hepatic enzyme elevations (53%). [cancernetwork.com]

  The male to female ratio is about 9:1.1 Patients often present with fever, fatigue, weight loss, and abdominal discomfort from hepatosplenomegaly, and sometimes, with jaundice because of liver involvement. [archivesofpathology.org]

• Liver Dysfunction

  The present case may shed light on of the mechanisms of liver dysfunction accompanying AITL. KEYWORDS: Angioimmunoblastic T cell lymphoma; Anti-mitochondrial M2 antibody; Liver dysfunction [ncbi.nlm.nih.gov]

  The condition may lead to liver dysfunction and even multi-organ failure. ATL – the main clinical presentations of this type include lymphadenopathy and hepatosplenomegaly. [symptoma.com]

  It is often associated with a systemic hemophagocytic syndrome and is characterized by high fever, skin lesions, lung infiltrates, jaundice, hepatosplenomegaly, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign prominent histiocytic [bloodjournal.org]

• Bone Pain

  A high calcium level in the blood (hypercalcaemia) may also be present which can cause confusion, bone pain and severe constipation. The lymphomatous form of ATLL presents with enlarged lymph nodes. [leukaemia.org.au]

• Skin Lesion

  After 35 years, the skin lesions spread to other acral sites, and a mass with the same histological features as the other skin lesions appeared on the nose. Multiple courses of chemotherapy led to stable disease. [ncbi.nlm.nih.gov]

  Acute ATLL is characterised by skin lesions similar to those found in mycosis fungoides or Sézary syndrome, enlarged lymph glands, high levels of calcium in the blood and bone lesions. [dermnetnz.org]

• Eczema

  Types & treatments Childhood eczema Adult eczema Insider secrets Types of hair loss Treatment for hair loss Causes of hair loss Hair care matters Insider secrets What is psoriasis Diagnosis & treatment Skin, hair & nail care Triggers Insider secrets What [aad.org]

  This condition can often be misdiagnosed or commonly mistaken for inflammatory, chronic skin conditions such as psoriasis or eczema. [hospitals.jefferson.edu]

  Diagnosis is difficult early in the course of this disease because it mimics several benign skin disorders, including eczema, psoriasis and contact dermatitis. [aafp.org]

  Early stages may cause small spots of red patches on the skin, which may look like eczema. [henryford.com]

• Night Sweats

  CASE PRESENTATION: A 74-year-old Caribbean-American woman presented with lymphocytosis, pruritus, and non-drenching night sweats. Bone marrow and peripheral blood analyses both confirmed the diagnosis of gamma-delta T-cell lymphoma. [ncbi.nlm.nih.gov]

  sweats, 10% weight loss A = absent B = present Extranodal sites are also designated M+ = marrow L+ = lung H+ = liver P+ = pleura O+ = bone D+ = skin and subcutaneous tissue Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also [surgpathcriteria.stanford.edu]

  Patients with this subtype of PTCL will frequently have constitutional symptoms (i.e., fevers, serious night sweats, and unexplained weight loss). [lymphoma.org]

  There are also other symptoms associated with the disease, including night sweats or fevers, unexplainable weight loss or lack of appetite, and in more severe cases, breathing problems, pain, distention and fatigue are also common. [lymphomanewstoday.com]

• Dermatitis

  View/Print Table TABLE 2 Differential Diagnosis of the Patch and Plaque Stage of Cutaneous T-Cell Lymphoma Psoriasis Atopic dermatitis/eczema Chronic contact dermatitis Chronic, nonspecific dermatitis Drug eruption Potential precursor lesions Poikiloderma [aafp.org]

  CTCL complicating severe atopic dermatitis. Dermatology 2009;218:168-71. [content.sciendo.com]

  The first biopsy showed an interface dermatitis-like pattern, revealing the deceptive features that early cutaneous infiltrates of this aggressive lymphoma may have. [ncbi.nlm.nih.gov]

• Skin Rash

  Skin rash and fatigue are also common symptoms in this form of ATLL. Smouldering ATLL develops slowly and presents with very mild symptoms such as a few lesions on the skin. [leukaemia.org.au]

  Clinical presentation The clinical spectrum includes high fevers, generalized lymphadenopathy, a skin rash, and hepatosplenomegaly. [radiopaedia.org]

  Smouldering ATL often causes a skin rash but some people don’t have any symptoms at all. You might just have a blood test that shows abnormal T cells in your bloodstream. [lymphoma-action.org.uk]

  Initial symptoms of ALCL can include fever, backache, painless swelling of lymph nodes, loss of appetite, itching, skin rash, and tiredness. ALCL can be either systemic (occurring throughout the body) or cutaneous (limited to the skin). [lymphoma.org]

• Facial Edema

  Nasal-type T-cell lymphoma – it affects nasal structures, with facial edema, nasal obstruction as common symptoms. They may present with bulky tumors which may be invasive. The cells may invade sinuses, oral cavity and orbit. [symptoma.com]

  Unlike typical HV, HVLL eruptions become more severe with age, presenting with marked facial edema and vesiclopapules followed by ulceration and crusting. Systemic symptoms, including high-grade fever and liver damage, are usually present. [frontiersin.org]

• Headache

  After 3 months, the patient began to present a sore throat, fever, and headaches. The computed tomography (CT) and magnetic resonance imaging (MRI) of her skull demonstrated nothing positive. [ncbi.nlm.nih.gov]

  短句来源 Results The main symptoms of nasal NK/T-cell lymphoma were nasal obstruction,epistaxis and headache,and presented with facial midline destructive lesion. 结果鼻腔NK/T细胞淋巴瘤以鼻塞、涕血和头痛为主要症状,表现为面部中线破坏性病变。 [dict.cnki.net]

  […] cause tummy (abdominal) pain a skin rash, which can vary from widespread, itchy red patches to lumps or skin tumours that can break down (ulcerate) and scab over high levels of calcium in your blood, which can cause sickness, diarrhoea, constipation, headaches [lymphoma-action.org.uk]

  The patient was stable for approximately 6 months; however, she then developed sudden onset of headache and neck pain. A lumbar puncture demonstrated 61 leukocytes per deciliter of cerebrospinal fluid (CSF), with 96% of these being lymphocytes. [jamanetwork.com]

• Dizziness

  However, side effects may include temporary low-grade fever, nausea, dizziness, and skin redness. Stem cell transplant A stem cell transplant is when your bone marrow is replaced with marrow from a healthy donor. [healthline.com]

  The most common include encephalopathy (57%), headache (44%), tremor (31%), dizziness (21%), aphasia (18%), delirium (17%), insomnia (9%) and anxiety (9%). Prolonged encephalopathy lasting up to 173 days was noted. [news.cancerconnect.com]

  Side effects Having this procedure does not hurt, but some people may feel a bit weak or dizzy during or after it. Afterwards your skin and eyes may be more sensitive to sunlight for about 24 hours. [cancerresearchuk.org]

  Patients with WM sometimes have elevated serum viscosity, or “thickened” blood, which may cause symptoms such as headache, blurry vision, dizziness, and shortness of breath. [cancer.net]

• Burning Sensation

  Other common side effects of treatment for CTCL include skin irritation and inflammation, redness, rash, itching, burning sensation (like sunburn), fatigue and depression. [curetoday.com]

  Tell your doctor if you have any side effects, such as redness or a burning sensation in the area. PUVA treatment PUVA (sometimes called photochemotherapy) is mainly used to treat skin symptoms during early CTCL. [macmillan.org.uk]

• Encephalopathy

  Also, we know that patients ages 65 and older are at a higher risk of experiencing the brain-related side effects of CAR T cell-related encephalopathy syndrome. [mdanderson.org]

  The most common include encephalopathy (57%), headache (44%), tremor (31%), dizziness (21%), aphasia (18%), delirium (17%), insomnia (9%) and anxiety (9%). Prolonged encephalopathy lasting up to 173 days was noted. [news.cancerconnect.com]

  AEs possibly related to treatment included 2 cases of acute kidney injury, one acidosis, 2 cytokine release syndrome, one fever, two hypotension, one hypertension, one delirium, and one case of encephalopathy, which resulted in death. [onclive.com]

Histological features of the affected tissue are checked with a peripheral blood examination and tissue biopsy. A more detailed evaluation is done using immunohistochemistry tests, flow cytometry, or molecular genetic tests. Classification of subtypes is based on a thorough hematopathology review. Imaging and bone marrow biopsy aids in staging of lymphoma. This is essential for assessing the extent of the disease and also to identify the characteristic features of the condition. Laboratory tests include full blood count, renal function test, liver function test, lactose dehydrogenase level, serum calcium and albumin level. Imaging techniques used in the diagnosis of different subtypes include chest X-ray, CT scan of chest, pelvis and abdomen. If central nervous system involvement is suspected, MRI and lumbar puncture may be suggested.

• Cytopenia

  Fever and cytopenia were the most common clinical presentations in both groups. The neoplastic cells varied from small and relatively monotonous cells to large pleomorphic cells. [ncbi.nlm.nih.gov]

  Hepatosplenic T-cell lymphoma – typical condition involves liver, spleen and bone marrow resulting in cytopenia and thrombocytopenia. Affected tissues will show sinusoidal infiltration of malignant cells. [symptoma.com]

  Common adverse events to the treatment of grade 3 or higher included cytopenias (94%) and infections (32%). [ajmc.com]

  Like HSTL, splenic marginal zone lymphoma and hairy cell leukemia often present with cytopenias and splenomegaly without lymphadenopathy. [archivesofpathology.org]

  The presenting feature of HSGDTL as per literature review include cytopenia, splenomegaly, fever, weight loss, fatigue and easy bruising. [intechopen.com]

• Leukopenia

  Grade 3/4 leukopenia occurred in 43 (23.4%) patients. Major non-hematological toxicities included nausea (n = 117, 63.6%) and vomiting (n = 66, 35.9%). [ncbi.nlm.nih.gov]

  Grades 3/4 adverse events (AEs) included hypophosphademia in 4 patients, leukopenia in 5, neutropenia in 9, and lymphopenia in 18 patients. [onclive.com]

  Routine laboratory studies revealed leukopenia and thrombocytopenia, at which time the patient was referred to our hematology clinic for further evaluation. [hematologyandoncology.net]

  Side effects were similar to those of other HDACi, and mainly included hematological adverse effects such as anemia, thrombocytopenia and leukopenia [20]. [futuremedicine.com]

• Human Papillomavirus

  According to this model, secondary immunodeficiency caused by HTLV-1 may induce the development of verrucous carcinomas; alternatively, the disease could be due to a correlation between HTLV-1 and the human papillomavirus (HPV). [ncbi.nlm.nih.gov]

Treatment modality differs with the type, stage, and organ involved. Standard methods of treatment include chemotherapy, radiotherapy, stem cell transplantation, and surgery. Patients with rare forms of lymphomas without any standard therapy may opt for clinical trials in the field. Chemotherapy follows three stages – remission induction, consolidation and maintenance. For some patients stem cell transplant may be suggested after high-dose chemotherapy. Combination therapy is suggested for PTCL and angioimmunoblastic T cell lymphoma, and if relapse occurs other chemotherapy regimens may be tried. Angioimmunoblastic T cell lymphoma may regress initially but usually becomes aggressive in time. Combination chemotherapy has a remission rate of 50%, but this condition is characterized by high relapse rates. Antiviral treatment is the first line of treatment for ATL. And if the condition progresses rapidly, combination therapy is used along with antiviral treatment.
Cutaneous T-cell lymphomas are treated with topical applications including steroid creams and chemotherapy lotions. Phototherapy and radiotherapy are also recommended to improve the symptoms on skin. Systemic treatments are suggested when the patient does not respond to topical treatment. Chemotherapy followed by stem cell transplant is suggested in some forms of lymphomas. T-LGL leukemia remain asymptomatic and may not need therapy initially. Treatment is usually based on complications like thrombocytopenia or neutropenia. Monoclonal antibodies and many other novel agents are being tried out in different clinical trials for controlling the symptoms. Anthracycline chemotherapy has given good results in the treatment of anaplastic large cell lymphoma.

Optimum therapeutic modality is not defined for many of the sub-types of T-cell lymphomas and they have a poor prognosis. The survival rates differ with the type of lymphoma. Prognosis may also be related to the stage of the disease as well as the type and extent of damage to the organ concerned [9]. Earlier diagnosis of the condition may be helpful in reducing mortality rate. In case of PTCL, more than 50% of the patients have an unfavorable prognostic index. Expression of certain cytotoxic molecules is associated with a lower response rate and a poor prognosis [10]. For example, expression of CCR4 is associated with inferior prognosis in the case of ATL. In ATL, the clinical subtype of the condition may also be an important prognostic factor.

The actual etiology of different types is not yet clearly defined. Number of factors like infections, genetic susceptibility, and immunodeficiencies are known to increase the risk of its occurrence. The risk of T-cell lymphoma and other NHLs increase with immunosuppressive therapy and organ transplants. An increased association between the occurrence of eczema and T-cell lymphomas is also reported. Etiology of adult T-cell lymphoma (ATL), a rare form of this condition, is reported to be linked to human T-cell lymphotropic virus type 1 (HTLV-1) infection [2]. A variant allele of tumor necrosis factor (TNF) is reported to be associated with an increased risk of the most common cutaneous T-cell lymphoma, mycosis fungoides. Some chemical or physical irritants that lead to the activation of oncogenes may also lead to this type of cutaneous T-cell lymphoma [3]. Lymphatic oncogenesis may also be caused by chromosome rearrangements, gene amplifications, and alteration of tumor suppressor genes.

Neoplasms of T/NK-cells form about 6% of lymphomas, while the majority of lymphomas are of B-cell type. Incidence rate of these lymphomas is reported to be 2.09 per 100,000 persons, much lesser than B-cell lymphomas [4]. Among the different subtypes of T-cell lymphomas, PTCLs have the highest incidence rates. Mycosis fungoides also has a similar incidence rate among the subtypes.

In general, the rate of incidence increases with age and these lymphomas are more common among males than in females. The rate may vary among different subtypes. T-cell lymphoblastic lymphoma is found to be more common among children under the age of 15 and among older adults above the age of 65. The age of occurrence may also differ among the subtypes. For example, mycosis fungoides begin in teenage while peripheral T-cell lymphoma not otherwise specified (NOS) develops only after 45 years of age. Incidence of PTCL is higher among Black population while angioimmunoblastic lymphoma is more common among Black males. Cutaneous T-cell lymphoma is found to be more prevalent among people engaged in specific industries that use machine cutting oils. The incidence of this subtype of lymphoma is found to be 6.4 in one million persons [5]. Incidence of ATL is higher in areas where HTLV infection is prevalent, while nasal-type T-cell lymphoma is associated with Epstein-Barr virus (EBV) infection.

Morphology and immunophenotype of these neoplasms are very complex and not complete defined. Thus, there is little information about the molecular pathogenesis of most these lymphomas. Alteration in antigen processing and presentation ensues genetic changes like inversions and deletions, leading to aggressive proliferation of the different types of T-cells.
T-cell acute lymphoblastic leukemia and lymphoma is caused by transformation of precursor T cells. The pathological and clinical manifestations of most of the T-cell lymphomas can be related to the expression of cytokines by the neoplastic cells [6]. In mycosis fungoides, clones of CD4+ memory T cells expand and lack the normal antigens seen on T cells [7]. They express cutaneous lymphocyte antigen (CLA), which helps in the adhesion of T lymphocytes to endothelial cells. Further to adhesion, these malignant cells exit the skin cells and move towards different regions.
Angioimmunoblastic T-cell lymphoma is related to expansion of regulatory T cells with CD25 and FoxP3 markers. These neoplastic, regulatory cells suppress immunity resulting in immune suppression, one of the characteristic feature of the condition [8]. ATL, the lymphoma associated with HTLV infection, shows increased production of a trans regulatory protein that increases the expression of T-cells resulting in neoplasm. Constitutive expression of proteins leads to neoplastic transformation of activated mature cytotoxic T cells in anaplastic large cell lymphoma (ALCL). Upregulated expression of CD30 by viral infections as in EBV and also result in lymphoproliferation.

In most of the cases, the risk factors associated with the condition cannot be changed. Hence, prevention of T-cell lymphomas is difficult. Preventing the risk factors like immune deficiency is the only way to avoid the occurrence of these neoplasms. Efforts to reduce the spread of HTLV-1 will help in controlling the incidence of T-cell lymphomas in areas where this virus is endemic.

T-cell lymphoma is a lymphoproliferative disorder that makes up about 15% to 20% of non-Hodgkin lymphoma (NHL). Most of the T-cell lymphomas are high-grade as they are aggressive in proliferation and localization. They are a group of very diverse nucleoid malignancies, uncommon in occurrence. These neoplasms are broadly categorized into precursor T-lymphoid neoplasms and mature T-cell and NK-cell neoplasms. Precursor T-cell lymphomas are more commonly seen among children and young adults. Mature T-cell lymphomas are commonly seen in adults and are more prevalent among men than in female. Mature T-cell lymphomas are further subdivided based on available information including morphology, immunophenotype, genetics and certain specific antigens [1]. The categories include

• Mature T-cell leukemia
• Nodal peripheral T-cell lymphoma (PTCL)
• Extranodal PTCL
• Cutaneous T-cell lymphoma

These are further subclassified into different types based on the clinical immunophenotypes and other characteristics. Treatment, prognosis and presentation varies between the different types of T-cell lymphomas. Most of the sub-types follow a protracted disease course and have poor prognosis.

T-cell lymphoma refers to the cancer of the lymphatic system in which the T-lymphocytes, one of white blood cells and an important component of the immune system, becomes malignant. They form about 15% of cancers called non-Hodgkin lymphoma. They are a heterogenous group of cancer and is divided on the basis of the cellular characteristics including shape and size of the cells. It may develop from any of the lymph nodes distributed in different parts of the body. They may develop from the immature or mature T-cells. Lymphomas of mature T-cells are usually seen in adults and is more common among men when compared to women. Those affecting immature T-cells mostly occur in children and young adults.
In most of the cases the actual cause of the lymphoma is not known. In some types lymphoma develops from virus infections. The most common symptoms of these lymphomas are weight loss, fever and night sweats. Swollen lymph nodes and rashes on the skin are also seen in many cases. The symptoms of the different types vary with the affected tissue and extent of damage. Diagnosis of the type of T-lymphoma is very important as treatment differs with the type of lymphoma. Various lab tests, imaging techniques and histological tests are used to identify the type of T-cell lymphoma. Biopsy is the most common method suggested to identify the lymphoma.
Treatment modalities recommended also differ from type to type. Most of T-cell lymphomas are very aggressive and thus early diagnosis and treatment is very important. Chemotherapy is the first line of treatment in many subtypes. In some cases a combination of different chemotherapy drugs are used to improve the symptoms. Stem cell transplantation is suggested in some cases. Radiotherapy and surgery are also used in the treatment depending on the type and extent of damage. In cases where viral infection is a cause, antiviral therapy is recommended. In general, T-cell lymphomas do not have a defined treatment modality and prognosis is poor.

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2. Mahieux R, Gessain A. HTLV-1 and associated adult T-cell leukemia/lymphoma. Rev Clin Exp Hematol. 2003;7(4):336-361.
3. Burg G, Kempf W, Cozzio A, et al. Cutaneous malignant lymphomas: update 2006. J Dtsch Dermatol Ges. Nov 2006;4(11):914-33.
4. Wang SS, Vose JM. Epidemiology and Prognosis of T-cell lymphoma. In: Foss F, ed.T-cell lymphomas. Newyork: Springer Science+Business Media; 2013.
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