Primary cardiac lymphoma is a rare diffuse B-cell non-Hodgkin's lymphoma that occurs primarily in the heart. It contributes to less than 1 % of all cardiac tumors and 2 % of all extranodal tumors. It often mimics other cardiac conditions like pericarditis and myocarditis. The clinical presentations are not specific, which results in their being frequently under-diagnosised. PCL typically affects people with compromised immune systems, such as individuals who have AIDS (acquired immunodeficiency syndrome), are taking immunosuppressive medication, or have had organ transplants in the past. However, PCL is also diagnosed in immunocompetent people. Diffuse large B-cell lymphoma is the most prevalent subtype of PCL, followed by T-cell lymphoma.
This article will describe the clinical presentation, diagnosis, and treatment options for PCL, emphasizing the most recent evidence-based guidelines and recommendations highlighting the difficulties in treating this uncommon and severe illness and the necessity for additional study to enhance PCL patient outcomes.
What Are the Clinical Presentations of Primary Cardiac Lymphoma?
Primary cardiac lymphomas (PCL) contribute to less than 1% of all cardiac tumors. Clinical presentation and diagnostic evaluation of PCL are difficult because of its rarity. If untreated, PCL has a terrible prognosis with a median survival of less than six months. Improving patient outcomes depends on early identification and rapid therapy initiation. Some common clinical manifestations of PCL are as follows:
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PCL can mimic both cardiac and non-cardiac diseases in their non-specific clinical presentation. The most typical PCL-presenting symptoms are fatigue, dyspnea, and chest pain. Palpitations, fever, night sweats, weight loss, and cough are other symptoms that patients may experience. These signs and symptoms are caused by the tumor's bulk effect, blood flow restrictions, and infiltration of the surrounding tissues.
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Up to 80 % of patients who have PCL report experiencing chest pain. The pain is often reported as acute, localized, and worsened by heavy breathing, coughing, or physical activity. The patients may also experience neck, back, arms, or shoulder pain. Another typical symptom of PCL that affects up to 60 % of patients is dyspnea. Usually, a tumor will impede the pulmonary veins, pulmonary artery, or superior vena cava, resulting in dyspnea.
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Another typical symptom of PCL is fatigue, experienced by up to 50 % of patients. Fatigue results from the tumor's bulk effect on the heart, which causes a decline in cardiac output. Palpitations are caused by arrhythmias brought on by cardiac involvement or by the tumor's impact on the conduction system.
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Patients with PCL may have a fever and night sweats since the tumor is linked to the B-symptoms. Fever, night sweats, and weight loss are called B-symptoms and are connected to lymphomas. Cancer cachexia, a metabolic condition characterized by weight loss, anorexia, and muscular wasting, may result in weight loss.
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Less than 20% of people with PCL experience a cough, which is a rare symptom. The compression of the trachea or bronchi by the tumor may cause a non-productive cough. Due to the tumor's impact on the surrounding structures, other symptoms, including hoarseness and dysphagia, may also manifest.
How Is Primary Cardiac Lymphoma Diagnosed?
A few of the diagnostic procedures that could be performed to detect PCL are as follows:
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Echocardiogram: An echocardiogram employs sound waves to produce precise pictures of the heart. Any abnormalities in the structure or operation of the heart, including the existence of tumors, can be seen during echocardiography.
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Magnetic Resonance Imaging (MRI): A strong magnetic field and radio waves are used in MRI to produce precise images of the heart. Any tumor's size and position can be better understood with an MRI.
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Computed Tomography (CT) scan: Any anomalies in the heart's anatomy, including tumors, can be found with a CT scan.
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Blood Tests: Blood tests may be performed to examine kidney and liver function and to look for indications of infection or inflammation.
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Biopsy: To perform a tissue biopsy, a small sample of the heart's tissue must be removed and studied under a microscope. This is the best technique to diagnose PCL.
What Are the Treatment Strategies for Primary Cardiac Lymphoma?
Due to its location, PCL can be challenging to diagnose and treat, necessitating a multidisciplinary approach. The PCL treatment plans affect the patient's age, general health, disease stage, and personal variables.
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One of the main PCL treatment options is surgery. Surgical excision might be curative if the tumor is small and localized. However, surgery might not be an option if the tumor has spread or is too big.
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Chemotherapy is a treatment that kills cancer cells throughout the body by using powerful medications. The type of lymphoma and the patient's general health is typically considered when choosing the PCL chemotherapy treatment. Prednisone, Doxorubicin, Vincristine, and Cyclophosphamide are among the medicines that are frequently utilized. Chemotherapy can be used as the main form of treatment before or after surgery.
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High-energy X-rays are used in radiation therapy to kill cancer cells. Radiation therapy can be utilized as the main form of treatment before or after surgery. Although fatigue, rashes on the skin, and nausea are possible side effects of radiation therapy, these are typically transient and can be managed with supportive care and medication.
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Targeted treatment may occasionally be utilized to treat PCL. Drugs that specifically target chemicals or pathways vital to the growth and survival of cancer cells are used in targeted therapy. For instance, Rituximab, a monoclonal antibody, may be used to treat B-cell lymphomas.
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Patients with advanced PCL have the option of a stem cell transplant. With a stem cell transplant, the patient's damaged bone marrow is exchanged for healthy stem cells to create new blood cells. Although stem cell transplantation is a difficult and potentially dangerous procedure, it may provide a chance for recovery in some circumstances.
Conclusion
In conclusion, a rare and aggressive form of lymphoma that affects the heart is called primary cardiac lymphoma (PCL). PCL is uncommon, making diagnosis and treatment difficult. However, with improvements in biopsy methods and medical imaging, it is now possible to diagnose PCL more accurately. Surgery is only used to treat PCL when there is an obstruction or emergency and is often combined with chemotherapy and radiation therapy. Due to PCL's rarity, medical professionals must maintain a high index of suspicion for the illness in individuals who arrive with cardiac symptoms. Patients with PCL can benefit from a multidisciplinary approach and cooperation between cardiologists, hematologists, and oncologists. Ultimately, improving the prognosis for people with PCL will require further study and breakthroughs in available treatments.
