Burkitt's lymphoma
โน๏ธ About
- ๐งฌ Monoclonal proliferation of B lymphocytes.
- โก Very aggressive form of non-Hodgkin lymphoma (<1% of B-cell lymphomas).
- ๐จโโ๏ธ First described by Denis Burkitt, Irish surgeon working in Africa (1950s).
- ๐ Often seen in children and young adults; can affect multiple organs.
โ๏ธ Aetiology
- ๐ฆ First human tumour proven to be associated with a virus: EpsteinโBarr Virus (EBV).
- ๐ฉธ Cancer of the lymphatic system with rapid doubling time.
- ๐งพ Most cases linked to translocations involving the MYC gene on chromosome 8.
๐งฌ Translocations
- t(8;14)(q24;q32) โ most common (70%).
- t(2;8)(p12;q24).
- t(8;22)(q24;q11).
๐ Types
- ๐ Endemic: Equatorial Africa; EBV-associated; often affects the jaw/face in children (4โ7 yrs).
- ๐ Sporadic: More common worldwide; abdominal and bone marrow involvement, adolescents/young adults.
- ๐งฉ Immunodeficiency-related: Linked to HIV/AIDS, post-transplant; often EBV-negative.
๐ฉบ Clinical Features
- ๐ถ Predominantly in children/young adults.
- ๐ฆท Endemic: jaw/face swelling, tooth displacement.
- ๐ก๏ธ Sporadic: abdominal masses, bowel obstruction, hepatosplenomegaly.
- ๐ง Possible CNS, kidney, ovary, or marrow involvement.
- ๐ฉธ Leukaemic phase: resembles acute lymphoblastic leukaemia (L3 morphology).
๐ง Differentials
- Diffuse large B-cell lymphoma (DLBCL).
๐ฌ Investigations
- ๐ Bloods: FBC, U&E, LFTs, Ca, LDH (raised in bulky disease).
- ๐งช Excisional biopsy = gold standard (starry-sky histology).
- ๐ Flow cytometry for immunophenotype confirmation.
- ๐ฅ๏ธ Imaging: CT CAP + PET for staging.
- ๐ง Consider lumbar puncture (CNS spread common).
๐ Management
- ๐จ Staging must be rapid โ therapy within 48h of diagnosis.
- ๐ช Surgical resection if large obstructive abdominal mass.
- ๐ High-intensity, short-duration combination chemotherapy:
โ CODOX-M/IVAC
โ BFM regimens
โ Rituximab often added.
- ๐ Supportive care: tumour lysis prophylaxis (allopurinol/rasburicase, hydration).
- ๐ง CNS prophylaxis with intrathecal methotrexate.
- ๐งฌ Stem cell transplantation: for relapse or refractory disease.
๐งพ Case Vignettes
Case 1 โ Endemic Burkittโs ๐ฆท
A 6-year-old boy from Uganda presents with a rapidly enlarging jaw mass and tooth loosening. Biopsy shows a starry-sky pattern and EBV positivity.
๐ Key point: endemic, EBV-driven, facial/jaw involvement.
๐ Management: urgent chemo ยฑ rituximab, CNS prophylaxis.
Case 2 โ Sporadic Burkittโs ๐
A 17-year-old male in the UK presents with acute abdominal pain and a palpable mass. CT shows ileocaecal mass with intussusception.
๐ Key point: sporadic type, GI involvement, emergency presentation.
๐ Management: surgery for obstruction, then intensive chemotherapy.
Case 3 โ Immunodeficiency-associated Burkittโs ๐งฉ
A 32-year-old HIV-positive man with poor ART adherence presents with rapidly progressive cervical lymphadenopathy and night sweats. Biopsy confirms Burkitt lymphoma, EBV-negative.
๐ Key point: HIV-associated, aggressive course.
๐ Management: HAART optimisation + intensive chemo.
๐ References