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Burkitt lymphoma.ppt
- 1. BURKITT’S LYMPHOMA Presenter: WANYAMA RONALD STEPHEN MBChB v Supervised by Dr. Tenywa.Emmanuel
- 2. Introduction •Burkitt lymphoma (BL) is a highly proliferative B cell non Hodgkin lymphoma. •BL is characterized by the translocation and deregulation of the MYC gene on chromosome 8. •Burkitt lymphoma is a childhood tumor but it is observed in adult patients as well Major forms, 1. The endemic (African) form and 2. The nonendemic (sporadic) form., 3. Immunodeficiency related
- 3. History of BL • first described by Denis Burkitt 50 years ago. • ‘Lymphosarcoma’ in 1958 in a series of 38 ugandan children. • Named Burkitt’s Lymphoma in 1960. • Records similar to BL as early as 1887 at Mengo Hospital by Dr Albert Cook.
- 4. Epidemiology • BL is more common in children worldwide from 2 to 16 years In Africa, the mean age is 7 years. Outside Africa, the mean age is 11 years. • Accounts for approximately 40% of childhood NHL 2 • Burkitt lymphoma is endemic in people living in Equatorial Africa. It is sporadic in residents of the United States • The endemic form is common (100 per 1 million children) • The Sporadic form is rare (1 to 2 per 1 million children) • Sex • The M:F - 2-3:1.
- 5. Burden in Africa • Mainly in the Lymphoma Belt (Burkitt D 1962) • More Epidemiologically related to Malaria endemicity: where average temp >15.5ºC and have rainfall > 500mm/year. • Highest NHL incidence ( over 7.5 per 100.000) and mortality ( over 5.7 per 100.000) rates are recorded in Eastern Africa
- 6. In Uganda • NHL – 42.2 /million in 1991–1995 to 67.9/million in 2002–2006,with 2/3 BL(Wabinga et al) • Nothern areas more than southern (Ogwang et al )- BL -1.8 /million children • BL -9.5/million in 1960–71, to 34.3 /million for the period 1991–97.(Orem et al)
- 7. Pathogenesis of BL • translocation of c-MYC at band q24 from Xsome 8 to the Ig heavy chain regions on Xsome 14, t(8;14). • Less frequently rearrangements translocate c-MYC to a position close to the antibody genes in xsome 2 or 22). • Proto-oncogene c-MYC is dysregulated and thereby causing proliferation of B-Cells
- 9. Role of EBV • EBV infection predisposes to c-myc translocation • EBV causes B-lymphocyte proliferation. • EBV may affect apoptosis and T-Cell Response • The eBLs, which are EBV positive, express CD21, whereas the vast majority of sBL are EBV negative and lack CD21 expression
- 10. Role of malaria • BL mapped to malaria holoendemicity • Malaria infection stimulates B-cell proliferation and depresses cytotoxic T-cell (CD 8+) function.
- 11. Other risk factors • HIV occurrence of BL seems to be higher amongst HIV positive adults, while the evidence of an association amongst children with eBL is still disputed. • The role of other possible risk factors such as low social exposure to pesticides and to other infections such as schistosomiasis and arbovirus remain to be elucidated
- 12. Pathological features in BL • Histology — BL tumor cells are monomorphic, medium-sized cells with round nuclei, multiple nucleoli, and basophilic cytoplasm • Cytologically,BL cells resemble the small non-cleaved cells within normal germinal centers • A "starry-sky" pattern is usually present, imparted by numerous benign macrophages that have ingested apoptotic tumor cells
- 13. Classification Morphologically the WHO classification distinguishes Morphologic variants •Typical BL: eBL and sBL •BL with plasmacytoid differentiation: more commonly among immune deficient children •Atypical Burkitt or Burkitt-like variant: Borderline between Burkitt's lymphoma and diffuse large B-cell lymphoma Clinical and genetic variants •- Endemic •- Sporadic •- Immunodeficiency-associated
- 15. Clinical features • BL is a rapidly growing tumor • The endemic (African) form usually presents as a jaw or facial bone,loosening of teeth, tumor that spreads to extranodal sites including the mesentery, ovary, testis, kidney, breast, and especially to the bone marrow and meninges
- 16. Clinical features • The nonendemic (sporadic) form usually has an abdominal presentation, most often with massive disease and ascites, involving the distal ileum, stomach ,cecum and/or mesentery. • Immunodeficiency-related cases more often involve lymph nodes; both these and sporadic cases may present with blood involved. • Burkitt-like tumors involve lymph nodes, the nasopharynx, and GI tract. these tumors have a high propensity to invade the bone marrow and central nervous system
- 17. Staging The National Cancer Institute (NCI) system – A - Single solitary extra-abdominal site – AR - Intra-abdominal, more than 90% of tumor resected – B - Multiple extra-abdominal tumors – C - Intra-abdominal tumor – D -Intra-abdominal plus one or more extra- abdominal sites
- 18. St. Jude / Murphy’s Staging
- 19. DIFFERENTIAL DIAGNOSIS • Diffuse large B cell lymphoma(DLBCL) • Lymphoblastic lymphoma • Mantle cell lymphoma • acute lymphoblastic leukemia
- 20. INVESTIGATION • CBC • RFTs and Serum electrolytes, uric acid, creatinine, calcium, phosphorus • lactate dehydrogenase, • LFTs • CXR • Bone marrow aspirate and biopsy • CSF-cytology, cell count, • Biopsy - histological Dx
- 21. Management • General management Prevent tumour lysis SDM • Rehydrate the patient • Allopurinol and Rasburicase
- 22. Specific management • Early 1980’s single agent Cyclophosphamide • Later Cyclophosphamide, Vincristine ,Methotrexate (COM) ,Cyclophosphamide, Vincristine ,Prednisolone (CHOP) • IT- MTX • 2nd line –cytarabine and adriamycin CODOX-M/IVAC (Magrath) regimen for Burkitt's lymphoma • CODOX-M (cycles one and three): • Cyclophosphamide,Doxorubicin ,Vincristine • Methotrexate. +IT MTX, cytarabine • IVAC (cycles two and four): Ifosfamide,Etoposide ,Cytarabine • +IT MTX
- 23. • Thank you for listening