II. Pathophysiology

  1. Aggressive mature B-Cell Non-Hodgkin's Lymphoma with rapid proliferation
  2. Myelocytomatosis (MYC) Oncogene translocation triggers dysregulated cell growth
    1. All forms of

III. Types

  1. Sporadic
    1. Most common in North America
    2. Represents 30-50% of Non-Hodgkin's Lymphoma in children (esp. boys age 10-12 years)
    3. Incidence: 3-4 per Million children per year in U.S.
    4. Predilection for ileocecal valve region
    5. EBV association is uncommon (<20%)
  2. Endemic
    1. Most common in equatorial Africa and parts of Papua New Guinea (central africa)
    2. More than 95% cases are associated with Epstein-Barr-Virus (EBV) positive (Mononucleosis)
      1. EBV Is thought to be a Cofactor in malignant transformation (chromosomal arm translocation, Oncogene)
  3. Immunodeficiency-Associated
    1. Most commonly associated with HIV/AIDS or post-organ transplant status
    2. May be EBV associated in up to 30-40% of patients

IV. Findings: Sporadic Burkitt Lymphoma

  1. Gastrointestinal symptoms and signs (most common)
    1. Abdominal Pain
    2. Abdominal Distention
    3. Nausea and Vomiting
    4. Gastrointestinal Bleeding
    5. Abdominal mass (may be palpable)
  2. B Symptoms (constitutional)
    1. Fever
    2. Night Sweats
    3. Weight loss

V. Labs

  1. Complete Blood Count
  2. Comprehensive Metabolic Panel
  3. Coagulation tests
  4. Markers of high tumor burden (risk for Tumor Lysis Syndrome)
    1. Lactate Dehydrogenase (LDH) increased
    2. Uric Acid increased
  5. Diagnosis
    1. Biopsy with histopathology and cytogenetics (including MYC translocation)

VII. Differential Diagnosis: Sporadic Burkitt Lymphoma

VIII. Management: Sporadic Burkitt Lymphoma

  1. Acute Abdominal Pain presentation management (e.g. surgery, Antibiotics)
    1. Small Bowel Obstruction
    2. Intussusception
    3. Bowel perforation
  2. Tumor Lysis Syndrome prevention
    1. See Tumor Lysis Syndrome
    2. Aggressive hydration
    3. Electrolyte correction (Hyperkalemia, Hypocalcemia, Hyperphosphatemia)
    4. Hyperuricemia management (e.g. Rasburicase, Allopurinol)
  3. Chemotherapy
    1. Highly responsive to short-course, intensive multi-agent Chemotherapy

IX. Complications

  1. Small Bowel Obstruction or Intussusception (with tumor as lead point in sporadic type)
  2. Bowel perforation
  3. Tumor Lysis Syndrome

X. Prognosis

  1. Cure rates approach 85-90% with early diagnosis and aggressive treatment
  2. Poor prognostic factors
    1. Advanced disease
    2. Delayed diagnosis
    3. Bulky tumor burden

XI. Resources

  1. Burkitt Lymphoma (Stat Pearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK538148/

XII. References

  1. Andriescu, Yen and Whitaker (2026) Crit Dec Emerg Med 40(10): 16-7
  2. Pagano (2009) Mediterr J Hematol Infect Dis 1(2):e2009030 +PMID: 21416007 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies