Primary central nervous system lymphoma

Disease Description (Causes and Symptoms)

Primary CNS lymphoma (Primary CNS lymphoma) is a non-Hodgkin lymphoma that arises primarily in the brain, spinal cord, cerebrospinal meninges, or eyes. It is usually a malignant lymphoma of B-cell origin (commonly diffuse large B-cell lymphoma), and its incidence increases in immunocompromised states (e.g., organ transplantation, acquired immunodeficiency syndrome). Lesions commonly occur in the deep regions of the brain (the frontal lobe, deep white matter, and periventricular areas) and grow rapidly, causing focal neurological symptoms and symptoms of increased intracranial pressure.

Main symptoms

• Personality changes, changes in cognition or consciousness, memory impairment
• Seizures (epilepsy)
• Headache, vomiting (signs of increased intracranial pressure)
• Focal neurological deficits: hemiplegia, decreased vision, facial nerve palsy, etc.
• Visual field impairment or decreased vision (when the eye is infiltrated)
• Symptoms progress relatively rapidly, over several weeks to several months

Treatment Methods

Core principles of standard treatment (key points from the latest guidelines)

• High-dose methotrexate (HD‑MTX)-based chemotherapy is the foundation of treatment. It may be used alone or in combination with cytarabine, rituximab (a targeted antibody), and other agents.
• Whole-brain radiotherapy (WBRT) increases tumor response, but may cause delayed cognitive decline (neurotoxicity), particularly in older patients, so it is mainly used as an adjunctive or selective treatment.
• Autologous stem cell transplantation (ASCT) may be considered in some patients to reduce the risk of recurrence after remission.
• If there is cerebrospinal fluid or ocular infiltration, cerebrospinal fluid testing and local treatment (e.g., radiotherapy or local chemotherapy) may be performed as needed.
• Symptom relief: steroids (to reduce edema and relieve symptoms), anticonvulsants (to control seizures), and management of intracranial pressure

Precautions and self-management

• Increased risk of infection during chemotherapy → Seek medical attention immediately if symptoms of infection such as fever, cough, or lower back pain occur
• Monitoring for methotrexate-related nephrotoxicity and hematologic toxicity is necessary (renal function, electrolytes, and bone marrow function)
• Cognitive changes may occur after radiotherapy (particularly in older adults)
• Consultation regarding vaccinations and immunotherapy is necessary during the treatment period

Complications (disease- and treatment-related)

• Persistent neurological deficits (motor, cognitive, and visual field impairment)
• Increased intracranial pressure, meningeal spread (positive cerebrospinal fluid findings)
• Treatment-related: bone marrow suppression, nephrotoxicity, infection, and radiation-induced cognitive decline

Prognosis

• The prognosis tends to be poorer than that of systemic lymphoma, and factors such as age, immune status, initial neurological function, and treatment response affect the prognosis

Checklist Before an Outpatient Visit (Including Example Questions)

Items/documents to bring

• List of medications currently being taken (including prescription drugs, over-the-counter medications, and herbal medicines)
• Previous medical certificates and tissue biopsy results (pathology reports), previous imaging (CT/MRI), and test results (blood tests, kidney function, etc.)
• Notes summarizing when symptoms began and how they have progressed (the type and frequency of seizures, changes in consciousness, characteristics of headaches, etc.)
• Immune-related medical history: HIV test results, history of organ transplantation or immunosuppressant use
• Records of allergies and previous adverse drug reactions

Key information to provide to the doctor during the outpatient visit

• Recent imaging (if possible, the original image files or CD)
• Previous biopsy results and treatment history (if applicable)

Example questions (for patients/caregivers)

• If there have been changes in symptoms, record and present specifically when the changes began and what form they took.
• If seizures occur, organize the symptoms and timing before and after the seizure, as well as the medication history (whether anticonvulsants are being taken).

General Example Questions to Ask the Doctor

• What is the most appropriate standard treatment for this disease? (Including whether high-dose methotrexate and combination therapy are appropriate)
• What side effects are expected from treatment, and how can they be managed?
• What precautions should be followed to reduce the risk of infection and complications during treatment?
• What is the risk of recurrence after treatment, and what is the schedule for follow-up tests (checkups)?
• What impact will treatment have on cognitive function and daily life (driving, employment, etc.), and what is the rehabilitation plan?

Examples of Disease-Specific Questions

• In my case (considering my age, immune status, and imaging findings), should a tissue biopsy (brain biopsy) be performed first, or should drug therapy be considered first?
• What are the advantages and disadvantages of whole-brain radiotherapy and autologous stem cell transplantation (ASCT) in my situation, and what strategy do you recommend?