Introduction:
Lymphomas are solid cohesive tumors (neoplasia) of the immune system. Lymphoma classically originates from lymphoid tissue. But in immunocompromised patients such as HIV, it develops in the central nervous system. In lymphomas, the hematopoietic precursor cells (immature white blood cells) from the bone marrow make a cohesive mass that settles down in various body areas. They have more adherent molecules that stick to each other. But leukemia has no adhesive molecules that are sticking together. Both diseases are treated as a separate category. But some lymphomas can go into the leukemic phase. The lymphomas are masses of neoplastic (cancer cells) of the lymphoid tissues that undergo several mutations over the years and lose their cohesiveness. So this cohesive mass spill into separate cells into the blood circulations if they lose their cohesiveness. Some leukemic cells start from a bone marrow metastasis in many tissues, and they enter into some lymphoid tissue and transform into a cohesive molecule like lymphoma. So some circumstances, the leukemic cells change into lymphomatous masses in lymphoid tissue. So lymphomas may go into the leukemic phase and vice versa. Lymphoma is always a lymphocyte-related disease. It can be T-cell or B-cell-related lymphomas.
What Are Angioimmunoblastic T-Cell Lymphomas?
The lymph system is made up of lymph fluid, and lymphoid organs like the spleen, thymus, bone marrow, and lymph nodes. The lymph system also consists of lymphatic vessels. The lymph vessels are tubular channels that drain fluid from different areas of the body to the bloodstream.
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The lymph nodes remove the bacteria and viruses and other foreign bodies present in the lymph fluid. The lymph nodes are present in the body at different parts of the body as groups or clusters.
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The lymphocytes are present in the lymph nodes that act against foreign bodies, bacteria, and viruses. There are two types of lymphocytes B and T lymphocytes.
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The B lymphocytes produce antibodies to kill the antigens and the T lymphocytes directly destroy the antigens.
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T lymphocytes are a type of white blood cell part of the immune system that develops from precursor hematopoietic stem cells from the bone marrow. They fight infections. They are involved in antigen or acquired specific immune responses.
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The lymphoblastic lymphomas are divided into T-cell and B-cell lymphoblastic lymphomas.
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90 percent of the lymphomas are T-cell lymphoblastic lymphoma. The T cells are produced from the thymus gland. It is present in the upper chest behind the sternum. B-cell lymphoblastic lymphomas are less common than T-cell lymphoblastic lymphoma.
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The non-Hodgkin’s lymphoma is divided into B and T cell lymphoma. T-lymphomas are a subtype of Non-hodgkin’s lymphoma and peripheral T-cell lymphoma. Angioimmunoblastic lymphoma occurs when the T-cell lymphocytes in the lymph nodes become cancerous.
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T-cell lymphomas are an aggressive form of lymphomas. Angioimmunoblastic lymphomas are one the common subtype of lymphoma but it is a rare type of T cell lymphoma.
What Are the Signs and Symptoms of Angioimmunoblastic T–Cell Lymphoma?
It causes symptoms that affect the whole body. AITL usually involves the bone marrow. Bone marrow is a spongy tissue inside the bone from the blood cells produced. Since the bone marrow is affected, it can lead to low levels of red blood cells (anemia) and platelets (thrombocytopenia). There can be a fluid build-up around the lungs (pleural effusion), which can cause breathing difficulties. It also affects the immune system, so it will be difficult for the patient to fight infections. In AITL, abnormal immune cells can produce many antibodies called immunoglobulins. In AITL, abnormal antibodies act against the normal healthy cells, which is called an autoimmune reaction. A lump in AITL is formed under the neck, armpit, or groin skin. A swollen lymph node causes this as it becomes full of cancerous lymphoma cells. The symptoms include:
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Swollen lymph nodes in many places.
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Fever, night sweats, and unexplained weight loss.
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Swollen spleen and liver make a bloated tummy.
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Thyroid problems.
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Skin rash.
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Itching.
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A low red blood cell count (autoimmune hemolytic anemia) can make the patient feel tired or short of breath.
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Low platelet levels (immune thrombocytopenia) make the patient bleed or bruise more quickly than usual.
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Painful and swollen joints.
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Inflammation of blood vessels.
What Is the Diagnosis of AITL?
A biopsy is taken. In a biopsy, a small part of the lymph node is removed and studied by a pathologist. The procedure is done under local anesthesia or general anesthesia. An excisional node biopsy collects an adequate amount of tissue to do the necessary testing for diagnosis. A bone marrow biopsy is required for diagnosis. In this procedure, the cells are collected from the bone marrow and tested by a pathologist. The chest X-ray images help to identify the condition.
A PET scan is done to understand the sites of disease in the body before the treatment starts. A computed tomography scan and some blood tests are done. Before initiating the treatment some baseline tests such as heart scan, kidney scan, breathing test, and vitals (temperature, blood pressure, and pulse rate are tested).
What Is the Treatment for Angioimmunoblastic T-Cell Lymphoma?
Many factors are taken into consideration before deciding on the treatment plan. The doctors will meet with a team of specialists called the multidisciplinary team approach. The standard first-line chemotherapy includes-
Hyper CVAD - The drugs are cyclophosphamide, Doxorubicin, Vincristine, Methotrexate, and Dexamethasone or (Cyclophosphamide, Doxorubicin, Vincristine, Dexamethasone) (CHOP).
BFM 2000 - It is used in young adults. The drugs include Prednisolone, Methotrexate, Vincristine, Asparaginase, Cyclophosphamide, Cytarabine, Dexamethasone, Doxorubicin, Etoposide, and Ifosfamide.
The treatment phases are:
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The induction phase includes multi-agent chemotherapy.
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Consolidation phase chemotherapy.
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Maintenance phase chemotherapy.
Lymphomas have the tendency to recur. After relapse, the patient is offered high-dose chemotherapy with stem cell transplantation.
Conclusion:
Lymphoma is a cancer of the lymphatic system. In the last few years, the treatment options have increased and improved tremendously rise the survival rate of the patient. Many people are alive for many years after diagnosis. If someone has the above-mentioned risk factors and symptoms it is better to consult an oncologist or general physician to protect their health.
