Published on Dec 18, 2019 and last reviewed on Feb 03, 2023 - 6 min read
Abstract
Lymphoma is a type of cancer that affects the infection-fighting lymphatic system of the body. Learn about its causes, symptoms, types, diagnosis, treatment, and prognosis.
It is a cancer that originates in the lymphocytes (a type of white blood cells which is the germ-fighting system of the body). This cells usually present in lymph nodes, spleen, tonsils,
bone marrow, and thymus gland like organs, which help the body to fight off diseases. The cancer cells can affect all the organs of the lymphatic system and other organs of the body. The most common organs that this cancer spreads to are the liver, lungs, and bone marrow.
The lymphatic system moves lymph fluid throughout the body with the help of lymph nodes and vessels. These lymph fluids contain white blood cells, which fight off infections and prevent them from spreading. The lymph nodes trap and destroy bacteria and viruses that try to spread through the blood.
Treatment for lymphoma is decided based on its type and severity. The treatment options include chemotherapy, immunotherapy, radiotherapy, and bone marrow transplantation. It can affect people of any age, but it is commonly seen in children and young adults (15 to 24 years).
The two main types of lymphomas are:
1) Hodgkin’s Lymphoma - Dr. Thomas Hodgkin, a pathologist, identified the cancerous cells in the 1800s, which is now known as Hodgkin’s lymphoma. Patients with this type of lymphoma have Reed-Sternberg (RS) cells, which are large cancerous cells. The subtypes include:
Nodular sclerosis Hodgkin’s lymphoma - It is the most common variety of Hodgkin’s lymphoma. It more commonly affects young adults. With the right treatment, this type is highly curable.
Lymphocyte-depleted Hodgkin’s disease - It is an aggressive and rare type of Hodgkin’s lymphoma. It only affects 1 % of the population, and more commonly patients with a compromised immune system. The prognosis is poor.
Lymphocyte-rich Hodgkin’s disease - It commonly affects men, and around 5 % of patients diagnosed with lymphoma are of this type.
Nodular lymphocyte-predominant Hodgkin’s disease - Otherwise called NLPHL, and affects almost 5 % of lymphoma patients. It affects people between 30 and 50 years of age, most commonly men.
Mixed cellularity Hodgkin’s lymphoma - Here, both lymphocytes and RS cells are present. It is a more common type of lymphoma and often affects older men.
2) Non-Hodgkin’s lymphoma (NHL) - This is the more common type. Reed-Sternberg cells are not seen in patients with this type of lymphoma. Most commonly, B or T cells of the immune system get affected. The subtypes include:
B-cell lymphoma - The most aggressive type of NHL is diffuse large B-cell lymphoma (DLBCL). It originates from abnormal B cells in the blood and grows rapidly. The prognosis depends on the stage of cancer, but if left untreated, it can be fatal.
T-cell lymphoma - It is not as common as the B-cell variant. Only 15 % of patients are diagnosed with this type of lymphoma.
Follicular lymphoma - This type of lymphoma originates in the white blood cells, and commonly affects people older than 60 years.
Burkitt’s lymphoma - It commonly affects immunocompromised individuals and is a rare type of lymphoma. It commonly affects children.
Mantle cell lymphoma - It is a rare and aggressive form of lymphoma. It is usually diagnosed at a later stage. Until then, it usually affects the gastrointestinal tract or bone marrow and is challenging to treat.
Lymphoplasmacytic lymphoma - Otherwise called Waldenstrom macroglobulinemia is a rare type of lymphoma, that commonly affects older adults.
Small lymphocytic lymphoma - It is a slow-growing type of B-cell lymphoma.
Generally, lymphoma does not cause symptoms in the early stages, and it is usually diagnosed accidentally when an enlarged lymph node is found during routine physical examination. A single or multiple nodes may get enlarged in the neck, chest, armpit, groin, and stomach.
As symptoms are not specific, it is often overlooked. Some of the common signs and symptoms include:
Pain in the bones.
Cough.
Tiredness.
Enlarged spleen.
Night sweats.
Skin rashes, which can itch.
Shortness of breath (dyspnea).
Abdominal pain.
Unintentional weight loss.
These symptoms can occur in many conditions, so lymphoma is often diagnosed only during the later stages. If you have persistent enlarged node since long time with weight loss, then consult your doctor to check for lymphoma.
Although the exact cause of lymphoma is not known, it is believed to be caused when lymphocytes undergo a genetic mutation. This genetic mutation is responsible for the rapid growth and multiplication of abnormal lymphocytes. Abnormal lymphocytes, unlike the normal types, do not die, which results in too many diseased lymphocytes in the lymph nodes. This results in the swelling of the lymph nodes, spleen, and liver.
The following factors can increase the risk of lymphoma:
Females are susceptible to some types and males are susceptible to develop other types of NHL.
People with immunocompromised conditions or those who take immunosuppressant drugs.
People who get infected with Epstein-Barr virus and Helicobacter pylori infection are at risk of developing lymphoma.
Chemical and radiation exposure.
A positive family history.
The stages of lymphoma are based on how far the cancer has spread. The stages are:
Stage 1 - One lymph node or one organ gets affected.
Stage 2 - Two or more lymph nodes are involved on the same side of the diaphragm.
Stage 3 - Two or more lymph nodes are involved on both sides of the diaphragm.
Stage 4 - Cancer affects an organ and spreads to lymph nodes located far from it and affect many organs.
You might be subjected to the following tests and procedures if your doctor suspects lymphoma:
Physical examination - To check for enlarged liver or spleen and lymph nodes in the neck, underarm, and groin.
Lymph node biopsy - A lymph node might be removed completely or partly for testing. It allows determining if lymphoma cells are present.
Bone marrow aspiration or biopsy - A is inserted into your hip bone in iliac crest, and a sample of bone marrow is collected. This sample is tested for the presence of lymphoma cells.
CT, MRI, or PET scan - These imaging tests are used to look for signs of lymphoma in other parts of your body.
The doctor will form a treatment plan depending on the type of lymphoma, its stage, the overall health of the patient, and the patient’s choice. The goal of treatment is to bring the disease into remission by destroying as many cancer cells as possible. The treatment options include:
1) Periodic checkups - If you do not have any signs or symptoms, or if you have a very slow-growing type of lymphoma, the doctor might wait for sometime before starting treatment. In the meantime, you have to go for periodic checkups.
2) Chemotherapy - Your doctor will prescribe drugs, which are either administered intravenously (IV) or orally, to kill rapidly-growing cancer cells.
3) Radiation therapy - Here, high-powered X-rays and protons are used to kill cancer cells.
4) Bone marrow or stem cell transplant - High doses of chemotherapy and radiation are used to kill cells in the bone marrow. After this, healthy bone marrow stem cells from a donor or your body are transplanted into the blood, which helps rebuild the bone marrow.
5) Biologic therapy or Immunotherapy - Here, drugs are used to stimulate the immune system to attack cancer cells.
6) Antibody therapy - Synthetic antibodies are injected into the blood, which respond to toxins produced by cancer cells.
7) Radioimmunotherapy - In this treatment option, high radioactive doses are introduced into B and T cancer cells to destroy them.
8) Steroids.
9) Surgery - Splenectomy (surgical removal of the spleen) or other organs are surgically removed after lymphoma has spread.
Prognosis depends on the stage and type of lymphoma. Not all types of lymphoma are curable, but some of them are. For some very slow-growing types, the prognosis is good even without treatment.
To know more about lymphoma, consult a cancer specialist online now!
Non-Hodgkin's lymphoma is a tumor of the lymphocytes, a type of white blood cell, which forms the part of the immune system of the body and plays a vital role in fighting against infection. Aggressive and Indolent are the types of non-Hodgkin's lymphoma, which involves B-cells or T-cells.
A tumor of the lymphatic system, which is a part of the immune system that fights against infection, is called lymphoma. Lymph nodes, spleen, bone marrow, and thymus gland comprises the lymphatic system. Hodgkin's and non-Hodgkin's lymphoma are the two main subtypes of lymphoma.
Lymphoma rash appears as a single or multiple, scaly patches, nodules, or plaques that are red or purple in color. These are similar to other dermatologic conditions like psoriasis or eczema, which usually occur in non-Hodgkin's lymphoma and are rare in Hodgkin's lymphoma. Their most common location is buttocks and thighs, which may also spread to other areas when the condition becomes severe. Under severe conditions, the plaques may become inflamed, raised, darken, and bleed.
Lymph node examination, lymph node biopsy, complete blood picture, bone marrow aspiration biopsy, and imaging tests like CT (computed tomography), MRI (magnetic resonance imaging), and positron emission tomography (PET) are the procedures that help in diagnosing lymphoma.
Hodgkin's lymphoma is one of the tumors with a high curable rate with a five-year survival rate of 88%, whereas the prognosis of non-Hodgkin's lymphoma depends on the type and extent of spread. The five-year survival rate of non-Hodgkin's lymphoma (NHL) is comparatively less at about 72%. Of the two types of non-Hodgkin's lymphoma, T-cell NHL are aggressive, and B-cell NHL are slow-growing.
Although the cause of lymphoma is unknown, it has been reported to be due to genetic mutation in the lymphocytes, which causes them to multiply rapidly and prevent the diseased lymphocytes from death, thereby increasing the number of ineffective lymphocytes in the blood. It leads to swelling of the lymph nodes, spleen, and liver.
Depending on the rate of spread and the type of lymphoma cells present they are divided into low-grade, intermediate-grade, and high-grade lymphoma. Low-grade lymphomas are slow-growing and most are asymptomatic for many years. After a period of five to ten years, they turn into aggressive ones. The intermediate-grade lymphomas spread at a fairly rapid rate without treatment, and more than half to three-fourth of people enter remission with treatment. In the case of high-grade lymphomas, the spreading rate is aggressive, but they respond to aggressive treatment and 50 to 75% of people enter remission.
Patients with low-grade lymphomas do not experience any symptoms except that there can be lymph node enlargement in some people. They become aggressive and exhibit more symptoms when they turn into high-grade lymphomas after five to ten years.
Most Hodgkin's lymphoma are curable and have a better prognosis, whereas non-Hodgkin's lymphoma are slow-growing yet are not curable; however, the prognosis of NHL is good.
Low-grade lymphomas are slow-growing and do not exhibit symptoms until they become aggressive. Sometimes, lymph node enlargement may be associated with few people. Once they become aggressive after 5 to 10 years, they start exhibiting severe symptoms.
Lymphomas contribute to about three percent of all cancers. Non-Hodgkin's lymphoma is highly prevalent in children compared to Hodgkin's lymphoma, contributing to more than 60% of pediatric lymphomas, and it is common in older adults. Hodgkin's lymphoma is common in people in their twenties or above the age of 55 years.
Lymphomas are completely curable depending on the type and the extent of the spread of lymphoma. Especially when they are in the initial stages, they have an excellent prognosis. The five-year survival rate of non-Hodgkin's lymphoma is 72%, and Hodgkin's lymphoma is 88%.
Lumps formed in lymphoma are usually painless, appear in clusters but can also be single, and are rubbery in texture. They are restricted to one body region like the neck or may involve several areas like the neck, armpits, and groin.
Lymphoma cannot be diagnosed with the help of a blood test, and a lymph node biopsy of the involved node is usually recommended. If the blood picture shows decreased white blood cells or platelets, then a bone marrow aspiration biopsy may be recommended to determine if the lymphoma is present in the bone marrow.
Lymphadenopathies caused by infections, sarcoidosis, amyloidosis, autoimmune disorders, and drug sensitivity reactions, benign lymphoproliferative disorders like Kikuchi's disease, Rosai-Dorfman disease, etc., and atypical lymphoproliferative conditions like Castleman's disease, lymphomatoid granulomatosis, and lymphomatoid papulosis can be possible mistaken for lymphoma.
Itching without any rashes is present in more than one-third of people who suffer from Hodgkin's lymphoma, whereas it is less common in non-Hodgkin's lymphoma. Itching occurs due to the release of cytokines that fight against tumor cells.
Recent studies have reported that people who undergo remission following treatment with Hodgkin's lymphoma experience weight gain.
Last reviewed at:
03 Feb 2023 - 6 min read
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Dr. Goswami Parth Rajendragiri
Pathology
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