Clinical features of very aggressive lymphomas

~10% cases of NHL; require prompt treatment.

Burkitt lymphoma

• Endemic BL: presents in childhood/adolescence in Africa with large extranodal tumours in jaw or abdominal viscera; 90% associated with EBV infection; aggressive but curable disease.

• Sporadic BL: often children, rare in adults (median age 31); presents with rapidly growing lymphadenopathy, often intra-abdominal mass arising from a Peyer's patch or mesenteric node; BM, CNS and blood involvement frequent; 30% associated with EBV infection; also associated with HIV infection; aggressive but curable disease in non-HIV-associated cases.

Lymphoblastic lymphomas:

• Young patients (median age 15 years; >50% of childhood lymphomas); share features with ALL; T-cell LBL more frequent (85%) and usually associated with thymic mass; 33-50% present with BM involvement; CNS involvement common; commonly progresses to ALL; aggressive but potentially curable in children.

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