Disease Description (Causes and Symptoms)
- Definition: Burkitt lymphoma is a highly malignant non-Hodgkin lymphoma originating from B cells and characterized by rapid growth. It is classified into endemic, sporadic, and immunodeficiency-associated types (e.g., HIV-associated).
- Causes/pathophysiology: Rearrangement of the c-MYC gene (e.g., t(8;14)) is often present, promoting cell proliferation. It is frequently associated with Epstein–Barr virus (EBV) infection, particularly in the endemic type, and the risk of developing it increases in immunosuppressed conditions (after transplantation, HIV, etc.). It occurs more commonly in children (approximately 3–8 years old) and is more common in boys.
- Main symptoms: A mass that can be felt suddenly due to extremely rapid tumor growth. Abdominal mass, abdominal pain, vomiting, intestinal obstruction or bleeding, anemia, and generalized weakness. Central nervous system (CNS) involvement (headache, vomiting, neurological deficits) is relatively common.
Treatment Methods
- Standard treatment principles: Early initiation of aggressive chemotherapy is the key to treatment, and in many cases intensive combination therapy is administered together with CNS prophylactic treatment.
- Systemic chemotherapy: Examples of high-intensity combination regimens include CODOX‑M/IVAC and hyper‑CVAD regimens (selected according to the center and the patient's condition).
- Targeted therapy/immunotherapy: The anti-CD20 monoclonal antibody rituximab is added to standard therapy when the disease is CD20-positive and contributes to improved prognosis.
- CNS prophylaxis/treatment: Intracranial/intrathecal methotrexate or cytarabine, or high-dose systemic methotrexate, may be used.
- When the tumor burden is very large or in cases of relapse/refractoriness: Autologous hematopoietic stem cell transplantation (autologous HSCT) may be considered.
- Adjunctive/supportive treatment:
- Prevention of tumor lysis syndrome (TLS): Aggressive fluid administration, uric acid-lowering medications (allopurinol or rasburicase), and monitoring of electrolytes and kidney function.
- Anti-infective measures: Consider the use of growth factors (G‑CSF), antibiotics, and antifungal agents in preparation for neutropenia. Symptomatic treatment such as transfusions and antiemetics may also be used.
- Fertility preservation counseling: Counseling about preservation methods is recommended for patients of reproductive age.
- Precautions / risk of complications / points to consider for self-care:
- Acute TLS (hyperuricemia, hyperkalemia, etc.) can rapidly become life-threatening, so monitoring during initial treatment while hospitalized is essential.
- Chemotherapy side effects such as bone marrow suppression (anemia, infection, bleeding), gastrointestinal problems, and hair loss may occur.
- When CNS involvement is present or prophylaxis is needed, be aware of complications related to lumbar puncture/intrathecal administration (such as headache).
- Vaccination during treatment, particularly with live vaccines, is often contraindicated; consult the attending physician.
- Long-term complications: Monitoring is needed for relapse after treatment, chronic respiratory, cardiac, and endocrine problems, and possible infertility.
Checklist Before an Outpatient Visit (Including Items to Bring and Example Questions)
- Items/documents to bring:
- List of currently used medications (including prescription drugs, OTC medications, and herbal medicines)
- Previous test results (blood tests, pathology reports, CT/MRI/PET images or reports)
- Past medical history (immune disorders, transplantation, HIV status, etc.) and allergy information
- Records of recent weight changes, fever, bleeding, and infection symptoms; notes on changes in pain or abdominal masses
- For women, the possibility of pregnancy (date of most recent menstrual period and whether pregnant)
- Items to confirm in advance at the outpatient visit:
- Whether recent blood test results are available (blood cell counts, electrolytes, kidney function, uric acid, etc.)
- The onset and progression speed of symptoms (e.g., whether the mass is growing rapidly)
- Example questions (questions that are useful to prepare before seeing the doctor)
- "Could the medications I am taking (or supplements) affect my treatment?"
- "Will I need to be hospitalized when treatment starts, or can it be provided on an outpatient basis?"
Examples of General Questions to Ask the Doctor
- "What are the results of my biopsy (immunomarkers, MYC gene testing, etc.), and how will they affect the choice of treatment?"
- "What is the expected treatment plan (regimen name, number of cycles, length of hospitalization), and how will side effects be managed?"
- "What measures are you planning to take to prevent tumor lysis syndrome?"
- "What are the recommendations for preventing infections and receiving vaccinations during and after treatment?"
- "How will long-term follow-up testing after treatment (surveillance for relapse) be conducted?"
Examples of Disease-Specific Questions
- "What were the results of my CD20/EBV/MYC tests, and why do you recommend specific treatments such as rituximab or high-dose methotrexate?"
- "The risk of CNS involvement (or actual involvement) has been confirmed. Which strategy do you recommend between intrathecal treatment and high-dose systemic treatment, and what side effects can be expected?"
(Note: The information above is general information, and the recommended diagnosis and treatment may vary depending on an individual's medical history and test results. Please consult the attending physician to determine a specific diagnosis and treatment plan.)