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Specialty scores for T Cell Lymphoma
T Cell lymphoma is a distinct type of lymphoma (cancer of the immune system and white blood cells). Lmyphoma can be of Hodgkin variety or non-Hodgkin variety. Hodgkin lymphoma differs with non-Hodgkin lymphoma in having a special kind of cell, the Reed-Sternberg cell. On the contrary, the body lacks Reed-Sternberg cells in non-Hodgkin lymphoma and damage on own tissues and organs are mediated by rapid proliferation of lymphocytes, a type of white blood cell that acts as an integral part of the immune system.
The body has two main types of lymphocytes that can cause lymphomas when control mechanisms over their proliferation fail: B Lymphocytes and T Lymphocytes. When T Cells keep on growing and multiplying rapidly, the body is labeled to be suffering with T Cell Lymphoma. T Cell lymphoma is of two broad types: Immature & mature. Immature T Cell Lymphoma consists of Precursor T-Lymphoblastic Lymphoma/Leukemia & mature T Cell Lymphoma (also known as Peripheral T Cell Lymphomas, comprising 15% of all NHL cases) consists of four distinct types, Cutaneous T Cell Lymphoma (CTCL), Extranodal T Cell Lymphoma, Nodal T Cell Lymphoma & Leukemic T Cell Lymphoma.
T Cell Lymphoma is caused by many different mechanisms, some of them still stand poorly understood. Viral infections can give rise to many types of T Cell Lymphomas, and prominent infections include HTLV-1 (Human T-cell lymphotropic virus type 1), EBV (Epstein–Barr virus, also known as Human Herpesvirus 4), Adenovirus & Parvovirus B19.
It may be caused in persons who are at increased risk due to presence of one or more risk factors, such as heavy alcohol intake, chewing tobacco, smoking, exposure to ultraviolet rays of sun, exposure to infrared waves, radiation therapy, celiac disease, exposure to toxins, chemicals exposures, and immunodeficiency. Some of the common genetic mutations include IDH2, TET2, DNMT3A, and more commonly, RHOA. However, the overall genetic basis of these cancers is still poorly defined.
Signs and symptoms:
Signs and symptoms vary according to the specific subtype of T-cell lymphoma. Cutaneous T Cell Lymphomas, such as Mycosis fungoides and Sezary syndrome, mainly manifest with patches of flat and scaly skin, erythematous and severely itchy rash, thickened skin plaques, subcutaneous lumps, nail and hair abnormalities, non-healing skin sores, and red skin bumps.
In nodal variety of T Cell Lymphoma, such as Peripheral T-cell lymphoma, not otherwise specified, Angioimmunoblastic T-cell lymphoma & Anaplastic Large Cell Lymphoma, involvement of lymph nodes is seen in the form of lymphadenopathy. Other features may include hepatomegaly, splenomegaly, weight loss, hemolytic anemia, and hypergammaglobulinemia. Constitutional symptoms are a hallmark in almost all types of T Cell Lymphomas, including fever, night sweats, chills, body aches, weight loss, malaise, loss of appetite, fatigue and lethargy.
Enteropathy associated T cell lymphoma classically presents with features of malabsorption syndrome, such as abdominal pain, weight loss, diarrhea, malnutrition, and lymphoma, such as swollen glands, fever, night sweats and weight loss, bundled together. In this type of T Cell Lymphoma, intestinal perforation is a fairly common complication, mandating urgent surgical intervention.
Peripheral T-cell lymphoma, unspecified (Nodal subtype of T Cell Lymphoma) commonly involves lymph nodes, liver and bone marrow. Angioimmunoblastic T-cell lymphoma (Nodal subtype of T Cell Lymphoma) commonly involves lymph nodes, liver, spleen and gives rise to pleural effusion. Anaplastic large cell lymphoma commonly involves mediastinum, pericardial and pulmonary nodules. Adult T-cell leukemia/lymphoma (Leukemic subtype of T Cell Lymphoma) commonly involves mediastinal and omental lymph nodes, leukemic meningitis and lytic bone lesions. Precursor T-cell acute lymphoblastic lymphoma (the Immature type of T Cell Lymphoma) commonly presents as mediastinal masses, lymphadenopathy and hepatopslenomegaly. In Anaplastic Large Cell Lymphoma, Primary Systemic Type, Gastrointestinal involvement is extremely common and features may include peritoneal lymphomatosis, ascites, omental masses, and mesenteric masses. The clinical manifestation in Extranodal NK/TCL, Nasal Type may include destructive nasal or midline facial tumor, palatal destruction, orbital swelling, or edema.
In some forms of T Cell Lymphomas, involvement of central nervous system (brain, spinal cord), gastrointestinal system (stomach, intestine), bones and lungs may occur, thus giving rise to signs and symptoms of blood in vomiting or stool or both, bowel perforation, pleural effusion, weakened bones with bony pain, chylothorax, pulmonary parenchymal disease, loss of consciousness and weakness. The constellation of thrombocytopenia, anemia, and leukopenia can be seen commonly.
Diagnosis of T Cell Lymphoma requires a comprehensive work up. The suspicion of disease should be made by the medical history itself. Further, accurate physical examination should be aimed to elicit particular clinical findings that support the diagnosis of T Cell Lymphoma, such as splenomegaly, hepatomegaly, skin lesions, lymphadenopathy etc. Routine lab tests must be carried out to examine any abnormalities in the blood cells, in the form of anemia, neutropenia, leucopenia, and thrombocytopenia. Since lymphomas have a tendency to involve bone marrow in a diffuse fashion (not specifically growing as a tumor, but mixing-in with other cells of the bone marrow), CT and PET scans are unable to detect specific involvement of bone marrow involvement. Hence, a bone marrow biopsy is very important diagnostic step to confirm the bone marrow involvement.
Tumor biopsy or skin biopsy may yield definitive diagnosis when histopathological examination is conducted and T Cell Lymphoma specific findings are observed. To find out the extent of the disease in the body, imaging studies are conducted, such as CT scan, MRI scan and PET scan. CT scan is the most commonly used imaging technique for pretreatment staging and for restaging of T Cell Lymphoma by performing assessment for lymphadenopathy and organ involvement. Evidence suggests that FDG PET has significant value in the assessment of tumor burden and response to individual treatment, facilitating optimization of appropriate treatment mode.
Management of T Cell Lymphoma involves chemotherapy, radiation therapy, autologous stem cell transplant, and surgery. Many patients respond to combination chemotherapy, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone); CHOEP (CHOP plus etoposide); or EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin). Approved drugs for the management of refractory or relapsed Peripheral T Cell Lymphoma are pralatrexate, gemcitabine, romidepsin, belinostat, and brentuximab vedotin. Other chemotherapy regimens that are used with variable success include ESHAP (etoposide, methylprednisolone, cytarabine and cisplatin) and DHAP (dexamethasone, cytarabine and cisplatin).
Skin-directed therapies are useful for peripheral cutaneous T Cell Lymphoma which corresponds to patch and limited plaque disease and include topical treatments such as corticosteroids, retinoids, imiquimod, topical chemotherapy, methotrexate, local radiation, photopheresis, and ultraviolet light (phototherapy). Mechlorethamine gel is U.S. FDA approved treatment for patients with mycosis fungoides, a type of cutaneous T-cell lymphoma (CTCL), who have received prior skin-directed therapy.
More advanced disease is commonly treated with strong and extensive radiation therapy, combination chemotherapy, and other therapies such as bortezomib, denileukin diftitox, alemtuzumab, liposomal doxorubicin, and vorinostat. Many treatments are experimental and at various stages of drug development including everolimus, lenalidomide, panobinostat, forodesine, APO866, KW0761, and others. Allogeneic stem-cell transplantation may benefit a population of patients who suffer with disease relapse after autologous transplantation.