Introduction:
Lymphomas are a cancer of lymphoid cells of the immune system. The two major lymphocytes are B cell and T cell lymphomas. The onset of the disease is very stormy. The patient's condition deteriorates very rapidly if treatment is not done appropriately. Acute lymphoblastic lymphoma is the most common malignancy in children. Immature or precursor lymphocytes or progenitor cells that form lymphocytes are called lymphoblasts. When an adult lymphocyte is antigenically stimulated it decides to multiply and the nucleus becomes something like a lymphoblast. T-cell lymphoma is a non-hodgkin’s lymphoma where the spread of the cancer is unpredictable and not in an orderly fashion. They develop in lymphoid tissues such as the spleen, lymph nodes, skin, gastrointestinal tract, liver, and others. The T cell lymphomas are seven percent of all non-hodgkin’s lymphoma. They grow aggressively or they are indolent (slow growth).
What Are CD8 + Cells?
CD 8 plus is a type of white blood cell in the blood. They are produced by the T cells of the thymus. They are called cytotoxic T lymphocytes.
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The CD8 plus cells help fight cancer and the germs that live within the cells. The range of CD8+ cells is 150 to 1000 per cubic millimeter.
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They are produced from the thymus and are expressed in the T cell receptors. They are crucial for immune defense because they fight against bacteria, viruses, and cancer cells.
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They secrete tumor necrosis factor (TNF-alpha and IFN-gamma) that has anti-tumour and anti-viral properties.
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CD8 plus cells kill the antigen by serial killing means they release the cytotoxic granules then move to a new target place and kill again. Hence called serial killers of the immune system.
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CD8 plus cells kill the virus-infected cells and they generate antiviral cytokines such as interferon-gamma. Likewise, they resist primary and secondary viral infections so the body. The CD8+ plus kills the cancer cells by recognizing the T-cell receptors of the specific antigens y the release of HLA-1 (human leukocyte antigen class I.
What Are Primary Cutaneous T-Cell Lymphomas?
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It is a group of blood cancers that causes symptoms like itchy skin and rash. Cutaneous T-cell lymphomas are non-hodgkin’s lymphoma cancers.
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T-cell lymphocytes mutate that multiple uncontrollably. Primary cutaneous T-cell lymphoma is a rare type of cancer of the white blood cells.
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They cause round patches on the skin. It is one of the non-hodgkin’s lymphoma. The exact cause of the condition is unknown.
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The mutations in the cell’s DNA make the cells grow more rapidly creating abnormal cells. Certain infections cause the bone marrow to create more abnormal lymphocytes. These abnormal T cells attack the skin. They cause skin redness, scaly round patches, and skin tumors.
What Is Primary Aggressive Epidermotrophic CD8+ Cytotoxic T-Cell Lymphoma?
Primary cutaneous aggressive epidermotrophic CD8(+) T cell lymphoma is a cytotoxic lymphoma that is characterized by aggressive and prominent epidermotropism of atypical CD8+ lymphocytes. They are very rare in all cutaneous primary T cell lymphomas with less than two percent incidence. The primary cutaneous T cell lymphomas account for 75 percent of all T cutaneous lymphomas. The rare type of primary cutaneous T-cell lymphoma is primary aggressive epidermotrophic CD8+ cytotoxic T-cell lymphoma. It has distinct clinical-pathological phases.
What Are the Stages of Primary Aggressive Epidermatropic CD8+ Cytotoxic T-Cell Lymphoma?
The stages are:
Stage 1 - Lymph nodes are normal. Red scaly patches, plaques, and bumps on the skin. In this stage, only the skin is affected. A small number of sezary cells in the blood.
Stage 2 -
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2A - Skin symptoms are the same as stage 1. Scaly patches, dry, red, plaques, and bumps on the skin that cover 80 percent of the skin. Lymph nodes are enlarged. One tumor or lesion on the skin is 1 cm or more in size. There are skin symptoms in the previous stages that have spread to the lymph nodes or blood. It spreads to other organs such as the lungs, liver, and bone marrow.
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2B - There are one or more tumors on the skin that are greater than 1 cm. The lymph nodes may or may not be affected. There are one or more raised tumors in the skin that do not contain lymphoma cells.
Stage 3 - The lymph nodes are affected or they are not affected. The lymph nodes are enlarged but do not contain abnormal cells.
Stage 4 -
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Stage 4A - The abnormal lymphocytes spread to the blood and lymph nodes. Numerous abnormal lymphoma cells are present in the bloodstream and the lymph nodes have abnormal lymphoma cells.
What Are the Symptoms of T-Cell Skin Lymphoma?
The T-cell skin lymphomas are patches of discolored, dry commonly found on the waist, buttocks, and shoulders. The patches are itchy. They look similar to dermatitis, psoriasis, and eczema. The symptoms of T-cell skin lymphoma are:
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Plaques are formed that are harder and thicker areas of skin.
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Large swellings are formed on the skin that is called nodules which ulcerate over time.
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Generalized reddening of the skin which is intensely itchy and scaly and dry.
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Cracked skin on the palms of soles and hands of the feet.
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Rash-like redness is present on the full body and is intensely itchy.
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Enlarged lymph nodes.
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Lumps are formed on the skin and they break open.
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Patches of skin look lighter in color than the skin surrounding them.
What Are the Diagnostic Tests for Primary Aggressive Epidermotrophic CD8+ Cytotoxic T-Cell Lymphoma?
The diagnostic tests include the following:
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Blood Tests - Cancer cells are found in the blood that is used to better understand the condition.
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Skin Biopsies - A small sample of skin is cut and diagnosed by a pathologist who determines whether it has cancer cells.
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Computed Tomography (CT) or Positron Emission Tomography (PET) Scan - Imaging tests are used to assess the spread of cancer.
What Is the Treatment for Primary Aggressive Epidermotrophic CD8+ Cytotoxic T-Cell Lymphoma?
The following are the treatment for aggressive epidermotrophic CD8+ cytotoxic T-cell lymphoma:
1. Phototherapy - It is a form of light therapy that uses ultraviolet, bright lights on the skin. Phototherapy treats skin conditions like eczema and psoriasis. Ultraviolet B or A are used in phototherapy after the application of medication that makes the skin cells more sensitive to phototherapy.
2. Radiation Therapy - X-rays are used to slow the growth of cancer cells or kill them.
3. Chemotherapy - Drugs are used to kill cancer cells or prevent their growth. They are Bexarotene and Interferons. Interferon-gamma is an immunologically active cytokine, folic acid metabolism inhibitor and histone deacetylase inhibitor (HDAC), and monoclonal antibody.
4. Immunotherapy - The treatment boosts the immune system with substances that are made in the lab or taken from one’s own body. The treatment uses the body’s immune system to fight against cancer cells.
5. Corticosteroids - They help control redness and itchiness.
6. Bone Marrow Transplant- This procedure replaces the abnormal or diseased bone marrow with healthy bone so the healthy donor bone marrow cells travel to the diseased bone marrow and starts generating healthy blood cells.
Conclusion:
The lymphomas tend to reoccurrence. During the time of relapse, they are treated with different doses of drugs along with radiotherapy. The survival rate of the individuals and the symptoms can be improved with early diagnosis and continuous treatment
