Burkitt’s lymphoma

Endemic BL

§   Equatorial Africa, PNG

Sporadic BL

Immunodeficiency associated BL

§   HIV

§   EBV in up to 40%

Sites of involvement

§   Extranodal sites +/- CNS involvement

§   Endemic – jaw and other facial bones in 50%

§   Sporadic

o        Abdominal masses (esp.ileo-coecal)

o        Breast involvement during puberty, pregnancy, lactation

§   A leukaemic phase may occur in bulky disease

§   Immunodeficiency – nodal involvement is common

Clinical Feature

§   Bulky disease due to short doubling time

§   Hypercalcaemia

§   BM involvement is a poor prognostic sign

§   Tumour lysis is common

Aetiology

§   EBV

§   In endemic BL, EBV is present in majority of neoplastic cells. The lymphoma is preceeded by prolongrd polyclonal B cell activation secondary to frequent infections (bacterial, viral, parasitic) which results in defective T cell regulation of EBV infected cells

§   <30 % in sporadic, 25-40% in immunodeficiency

Morphology

§   Multiple basophilic nucleoli

§   Basophilic cytoplasm with lipid vacuoles

§   Very high proliferation rate (mitotic figures)

§   ‘starry sky’ – macrophages that have ingested apoptotic cells

Immunophenotype

§   B-cell antigens. BCL-6 and CD10

Genetics

§   T(8;14) = translocation of MYC

Treatment

§        Combination chemo including drugs with high CNS penetration

§        50-80% cure rate

§        CODOX-M/IVAC

§        Cyclophosphamide

§        Doxorubicin

§        High dose MTX/ ifosfamide

§        Etoposide

§        High dose cytarabine

§        Intrathecal prophylaxis

§        Dose adjusted EPOCH – rituximab

§        Dunleavey et al 2006

§        CR of 100% at median FU of 28 months in 19 patients (HIV +/-)

§        Etoposide

§        Doxorubicin

§        Vincristine

§        Cyclophosphamide

§        Oral prednisolone

§        Rituximab

§   Filtration for renal failure

§   Rasburicase to prevent uric acid nephropathy