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Case 227 - Discussion

Uploaded: 2011-10-18, Updated: 2011-10-18

 

Burkitt Lymphoma

CLINICAL FEATURES

  • Epidemiology:

    • Endemic: tropical Africa, most common childhood malignancy, peak 4-7 years of age, involves jaw or abdomen, 95% EBV positive.

    • Sporadic: mostly in children or young adults. 30% of childhood lymphomas

    • Immunodeficiency-associated: HIV.

  • Locations:

    • Endemic: jaw and other facial bones in 50% cases, also distal ileum, cecum and omentum.

    • Sporadic: mostly in ileocecal area.

    • Immunodeficiency-associated: HIV.

  • May present as B cell acute lymphoblastic leukemia FAB L3 (Burkitt’s leukemia). Call "Burkitt’s leukemia" if tumor cells present in blood and diffuse marrow involvement at diagnosis.

  • Marrow involvement of 15-30%.

MICROSCOPIC FINDINGS

  • Diffuse infiltration of monomorphic, medium-sized cells with starry sky pattern.

  • Tumor cells:

    • Abundant basophilic cytoplasm. Some times "squared off" boarders.

    • Non-cleaved round nuclei with finely clumped and dispersed chromatin.

    • Multiple basophilic medium sized, paracentrally situated nucleoli.

  • Imprints demonstrate deeply basophilic cytoplasm with lipid vacuoles.

IMMUNOPHENOTYPE

  • Positive: CD10, CD79a, CD19, CD20, Ki-67 (almost 100%), BCL6, CD38

  • EBV positive in endemic African cases and AIDS cases.

  • Negative: BCL2, CD5, CD23, TdT.

CYTOGENETIC STUDIES

  • C-myc translocations, very characteristic but not specific:

    • t(8;14)(q24;q32): c-myc and IgH, 75%.

    • t(2;8)(p12;q24): Ig Kappa and c-myc, 15%.

    • t(8;22)(q24;q11): c-myc and Ig Lambda, 10%.

TREATMENT AND PROGNOSIS

  • Highly aggressive but potentially curable. Curable with aggressive therapy in 60% cases.

REFERENCES: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Fourth Edition. 2008.