<aside> 💡 TdT-, sIg+, CD10+, BCL6+, CD20+, Ki-67+ (≥95%), BCL2-, simple karyotype with MYC rearrangement as sole abnormality.

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Epidemiology

• Accounts for some 2% of all lymphomas.
• Occurs in the first two decades of life.
• More common in males.

Subtypes

Endemic BL

• Occurs in equatorial Africa and Papua New Guinea.
• Peaks in incidence in 4-to 7-year–old children.
• Predominantly a male disease.
• EBV is virtually always detected.
• Commonly present with jaw and other facial disease, and other extranodal sites of involvement include the ileocecum, gonads, kidneys, and breasts.
• Cures were occasionally achieved with single-agent cyclophosphamide.

Sporadic BL

• Presents worldwide and is the most common variant in the Western world.
• Typically affects children and young adults.
• More commonly observed in boys.
• EBV+ in 25-40% of cases.
• The ileocecal area is the most common site of disease involvement.
• Overall EFS of 65% at 2 years in UK.