BURKITT LYMPHOMA
-
Features:
30% of childhood NHL’s
Mature B cell lymphoma
Very aggressive
Responds well to intensive chemotherapy -
Types:
African
Sporadic
HIV associated -
Morphology:
High mitotic index
Contains numerous apoptotic cells
Abundant clear cytoplasm
Starry sky pattern of cytoplasm -
Immunophenotype:
Express surface IgM, CD19, CD20, CD10, BCL6
Fails to express anti-apoptotic protein BCL2 -
Molecular pathogenesis:
Associated with translocations of c-MYC gene on chromosome 8.
Translocation partner is usually IgH locus -
Clinical manifestations:
Extranodal sites
Endemic: Mass involving mandible
Sporadic: Mass involving ileocecum and peritoneum -
Treatment:
Intensive combination chemotherapy regimens
High doses of cyclophosphamide
Prophylactic therapy to CNS