Disease Description (Causes and Symptoms)
- Definition summary: Burkitt lymphoma is a highly malignant non-Hodgkin lymphoma originating from B cells. It has a very high proliferation rate and is a rapidly growing tumor. It is characterized by a “starry-sky” appearance on cytology and translocations of the MYC gene (t(8;14), t(2;8), t(8;22)).
- Causes/associated factors:
- Impaired regulation of cell proliferation due to MYC gene translocation.
- Epstein–Barr virus (EBV) infection: strongly associated with the endemic form. It is also associated with immunosuppression (e.g., HIV and immunosuppression after transplantation).
- The exact single cause is unknown.
- Pathophysiology:
- Because of the very high cell proliferation rate (Ki‑67 ≒ 100%), a large tumor burden, cell necrosis and hemorrhage, and central nervous system (CNS) involvement may develop within a short period.
- Main symptoms:
- Children and adolescents: acute abdominal pain, an abdominal mass (which may cause intestinal obstruction or intestinal ischemia), vomiting, weight loss, anemia, and signs of bleeding.
- Adults (sporadic form): involvement of abdominal organs (such as the ileum and peritoneum) is common.
- Face and jaw (endemic form), enlarged lymph nodes, fever, night sweats, and fatigue.
- With CNS involvement: headache, vomiting, neurological deficits, and altered consciousness.
Treatment (Standard Treatment, Medications, Procedures, Lifestyle Management, and Precautions)
- Active and rapid treatment is required after confirmation of the diagnosis (because of the rapid progression).
- Overview of standard treatment:
- Intensive (high-intensity) combination chemotherapy is standard. In children, LMB-based regimens are used; in adults, CODOX-M/IVAC, Hyper-CVAD, or intensive combination regimens are used. Rituximab (anti-CD20) is combined in most cases when the disease is CD20-positive to improve the survival rate.
- CNS prevention and treatment: CNS prophylaxis (or treatment) is performed using intrathecal (IT) methotrexate, cytarabine, and other agents, together with high-dose systemic methotrexate.
- Hematopoietic stem cell transplantation: considered in patients with relapse or refractory disease. Autologous (autologous hematopoietic stem cell transplantation) or allogeneic transplantation is decided on an individual basis.
- Surgery/radiation: limited except for tissue biopsy for diagnosis. Surgical treatment may be required for complications such as intestinal obstruction or bleeding.
- Supportive care and side-effect management:
- Prevention of tumor lysis syndrome: fluid administration, uric acid-lowering drugs (Allopurinol or Rasburicase), electrolyte monitoring (potassium, phosphorus, and calcium), and attention to kidney function.
- Infection prevention and management: use of antibiotics, antifungal agents, and antiviral agents; growth factors (G‑CSF) may be considered in cases of neutropenia.
- Transfusions (for anemia or bleeding), antiemetics, and nutritional management.
- Fertility preservation counseling (sperm and egg preservation counseling is recommended before chemotherapy).
- Precautions and risk of complications:
- Delaying rapid treatment worsens the prognosis. Hospitalization and inpatient treatment are common.
- Complications: tumor lysis syndrome, heart failure (with certain medications), severe bone marrow suppression, infection, neurological complications, intestinal perforation, and bleeding.
- Prognosis:
- In children and adolescents, active treatment results in a high cure rate (often reported to be over 80%). In adults, the prognosis varies more depending on the indication, stage, CNS involvement, and underlying disease.
Checklist Before an Outpatient Visit (Including Items to Bring and Example Questions)
- Items to bring:
- A list of all medications currently being taken (prescription drugs, over-the-counter drugs, herbal medicines, and supplements)
- Records of allergies (medications, foods, etc.)
- Recent test results (blood tests, imaging tests, etc.) or related reports
- A record of symptom changes (when they began, how quickly they progressed, weight changes, fever, bleeding, changes in bowel movements, etc.)
- Contact information for a caregiver or designated contact person
- Items that are useful to record during medical visits:
- Presence of fever and temperature records, urine output, gastrointestinal symptoms (vomiting, diarrhea, abdominal pain), and neurological symptoms (headache, visual disturbances, weakness)
- Precautions before an outpatient visit:
- If fever or acute symptoms (acute abdominal pain, difficulty breathing, significant bleeding, etc.) occur, visiting an emergency room is recommended.
- Regular blood tests, kidney function tests, and electrolyte tests are necessary.
- Example questions (to note down for the doctor):
- "What tests should be checked during this visit, and what imaging tests are scheduled?"
- "Are there any tests or vaccinations I need to prepare for before starting treatment?"
Examples of General Questions to Ask the Doctor
- What are the main side effects of this medication (or treatment), and how should I respond if they occur?
- What is the treatment plan and expected treatment duration based on my stage (and test results)?
- What measures are taken to prevent tumor lysis syndrome? Is inpatient treatment necessary?
- How should I adjust my daily activities (meals, exercise, work, and school) during treatment?
- What options should I consider to preserve fertility?
Examples of Disease-Specific Questions
- In my case (including CNS test results and whether there is bone marrow involvement), is CNS prophylaxis (IT injection or high-dose methotrexate) necessary? How many treatments will be needed and over what period?
- How do the presence or absence of a MYC translocation and EBV status affect treatment selection or prognosis? Are additional genetic or molecular tests necessary?
(Note: This summary is intended to provide general information about diagnosis and treatment. Specific diagnosis and treatment plans should be determined through consultation with the treating doctor.)