Burkitt lymphoma.ppt

Burkitt lymphoma (BL) is a very fast-growing type of cancer. It is a form of B-cell non-Hodgkin's lymphoma. Signs and symptoms may differ depending on the form of BL and the organs or body systems involved. When it spreads, weakness and fatigue often develop. Lymphoma cells may build up in the lymph nodes and other organs, causing swelling. Central nervous system involvement is possible with all forms of BL, particularly when there is advanced-stage disease. The exact cause of BL is not known. EBV infection appears to play a role in some cases. While acquired (not inherited) genetic changes involving the MYC gene and other genes are present within BL cancer cells, it is unclear what causes these genetic changes to occur.

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 patientsas 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

14. Overview of characteristics

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
•2
nd
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