Lymphoma
Introduction
Lymphoma is a type of cancer that originates in the lymphatic system, a network of vessels, lymph nodes, and organs such as the spleen and bone marrow that helps fight infections by producing and circulating lymphocytes, which are white blood cells crucial for immunity.
In simple terms, lymphoma occurs when these lymphocytes grow uncontrollably, forming tumors that can spread throughout the body. Unlike solid tumors, lymphoma often presents as swollen lymph nodes but can affect almost any organ.
There are two main categories of lymphoma:
- Hodgkin Lymphoma (HL): Accounts for about 10–15% of cases. It is more common in young adults and has excellent cure rates in many patients.
- Non-Hodgkin Lymphoma (NHL): A broader group accounting for approximately 85–90% of cases. It includes subtypes such as diffuse large B-cell lymphoma (DLBCL), follicular lymphoma, and Burkitt lymphoma. NHL varies widely in behavior, ranging from slow-growing to rapidly progressing disease.
Risk factors for lymphoma include weakened immunity, such as that associated with HIV infection or organ transplantation, certain infections such as Epstein-Barr virus (EBV), autoimmune diseases, exposure to certain chemicals such as pesticides, and a family history of lymphoma.
Clinical Presentation
Lymphomas can sometimes resemble infections such as tuberculosis or other conditions, which may delay diagnosis. The most common presentation is painless swelling of lymph nodes in the neck (cervical), armpits (axillary), or groin (inguinal).
Other symptoms may include fever above 38°C, night sweats that soak clothes, and unexplained weight loss. These symptoms may be associated with more advanced disease.
Some patients with Hodgkin lymphoma may experience itching, including itching that occurs after alcohol consumption. In advanced stages, symptoms such as chest pain, facial swelling, abdominal pain due to an enlarged spleen (splenomegaly), anemia, and easy bruising may occur.
In aggressive forms of NHL, such as DLBCL, lymph nodes may grow rapidly. Other symptoms can include abdominal pain, skin rash, headache, or seizures.
Indolent forms of NHL, such as follicular lymphoma, may be largely asymptomatic, with easy fatigability being one possible symptom. In some cases, lymph nodes may wax and wane in size over time.
Lymphoma should be considered when lymph node swelling persists for more than four weeks, particularly when associated with symptoms such as fever, night sweats, weight loss, or anemia.
Diagnosis
Diagnosis of lymphoma requires tissue confirmation, while imaging and laboratory investigations help determine the extent of disease and guide treatment decisions.
Laboratory investigations may include:
- Complete blood count (CBC)
- Lactate dehydrogenase (LDH)
- Beta-2 microglobulin
- HIV and hepatitis B virus (HBV) serology
- Flow cytometry
PET-CT is an important imaging investigation used for staging lymphoma. Removal of the entire lymph node, known as an excisional biopsy, is often required for confirmation, followed by immunohistochemistry to determine the specific lymphoma subtype.
Molecular testing may also be advised depending on the type of lymphoma and may provide additional information about prognosis and treatment options.
Management of Lymphoma
Choosing the appropriate therapy is important for achieving the best possible treatment response and outcomes. The type and combination of therapies are decided by the oncologist based on factors such as the patient's stage of disease, lymphoma subtype, age, overall health, and comorbidities such as diabetes, hypertension, or heart disease.
Hodgkin Lymphoma
For Hodgkin lymphoma, treatment may include the ABVD protocol, while escalated BEACOPP may be considered in selected high-risk cases. Involved-site radiation therapy may be used in certain early-stage cases.
For relapsed Hodgkin lymphoma, targeted therapy such as brentuximab vedotin, an anti-CD30 monoclonal antibody, may be considered along with autologous stem cell transplantation in appropriate patients.
Non-Hodgkin Lymphoma
For aggressive NHL such as DLBCL, the R-CHOP protocol is commonly used. For indolent NHL such as follicular lymphoma, an oncologist may recommend observation or watchful waiting in selected patients.
Intensive chemotherapy protocols may be considered for aggressive lymphomas such as Burkitt lymphoma. Allogeneic stem cell transplantation may be an option for selected patients with relapsed NHL.
Targeted Therapy and Immunotherapy
Advances in lymphoma treatment have introduced targeted therapies and immunotherapies alongside conventional chemotherapy. Targeted medicines such as ibrutinib or acalabrutinib may be used for selected patients with mantle cell lymphoma.
Other targeted treatments, including venetoclax, may be considered for specific lymphoma subtypes depending on the patient's disease characteristics.
Immunotherapies have also demonstrated activity in relapsed or refractory Hodgkin lymphoma. One important development for selected patients with relapsed or refractory NHL is CAR-T cell therapy. In this treatment, the patient's T cells are genetically engineered to express chimeric antigen receptors that recognize specific antigens, such as CD19, on cancer cells.
Follow-Up Care
After completion of therapy, long-term follow-up is required to monitor for recurrence, second primary cancers, and late toxicities associated with chemotherapy and radiation therapy.
Patients should remain alert to symptoms that could potentially indicate recurrence, including:
- Headaches
- Convulsions or seizures
- Shortness of breath
- Chronic cough
- Bone pain
- Abdominal pain or distension
- Jaundice
- Unexplained weight loss despite adequate food intake
A general follow-up schedule may involve checkups every three months during the first two years, every six months between years three and five, and annually thereafter. The exact schedule should be individualized by the treating oncologist.
Lifestyle modifications may also be advised, including quitting tobacco and limiting alcohol consumption, maintaining a healthy weight, regular physical activity, consuming plenty of fruits and vegetables, and limiting excessive intake of red meat.
Preventive measures such as appropriate vaccination, control of HIV infection where applicable, and safe handling of pesticides can also contribute to reducing certain health risks.
Multiple Myeloma
Introduction
Multiple myeloma (MM) is a type of blood cancer that affects plasma cells, a type of white blood cell produced in the bone marrow that is responsible for making antibodies to fight infections.
In multiple myeloma, these plasma cells become cancerous and multiply uncontrollably, crowding out healthy blood cells. This can lead to weakened immunity, anemia, bone damage, and kidney problems.
Multiple myeloma can be thought of as a malfunctioning antibody-producing factory: instead of producing useful antibodies, abnormal plasma cells produce excessive amounts of an abnormal protein known as an M protein, which can contribute to complications.
Multiple myeloma primarily affects older adults, although younger people can also develop the disease. It may remain unnoticed until symptoms appear, and some patients are diagnosed after abnormalities are detected on routine blood tests.
Risk factors include older age, male sex, family history, obesity, radiation exposure, and conditions such as monoclonal gammopathy of undetermined significance (MGUS), which is a precursor condition that can progress to multiple myeloma in a subset of individuals.
Advances in treatment have significantly improved outcomes for many patients with multiple myeloma. Modern treatment approaches include chemotherapy, targeted medicines, immunotherapies, and stem cell transplantation.
Clinical Presentation
Symptoms of multiple myeloma can develop gradually and may sometimes resemble conditions such as aging-related problems or arthritis. Some patients may have no symptoms initially and may be diagnosed incidentally.
Common clinical features include:
- Bone pain: Particularly in the back, ribs, or hips, which may occur because of bone damage, fractures, or plasmacytomas.
- Severe sudden pain: Sudden severe back pain may indicate spinal cord compression, which requires urgent medical attention.
- Fatigue and weakness: Often associated with anemia caused by reduced production of healthy red blood cells.
- Shortness of breath and pale skin: These may occur as a consequence of anemia.
- Recurrent infections: Abnormal plasma c