This document provides information about Burkitt lymphoma, a type of non-Hodgkin lymphoma. It defines Burkitt lymphoma and discusses its epidemiology, causes, clinical presentation, investigations, staging, treatment, and prognosis. Burkitt lymphoma is an aggressive high-grade B-cell lymphoma that is endemic in equatorial Africa and associated with Epstein-Barr virus infection and malaria. It commonly affects children and presents with jaw swelling or abdominal masses. Treatment involves chemotherapy with cyclophosphamide, vincristine, methotrexate, and intrathecal chemotherapy, which can cure over 90% of cases when caught early. Prognosis depends on tumor burden and ability to reduce tumor size.

1.  Presenter:
Dr Opiro Keneth,
 Date: 6th July 2012

2.  Introduction
 Definition
 Epidemiology
 Aetiology
 Clinical Features
 Investigations
 Staging
 Treatment
 Prognosis

3.  Lymphomas are malignant proliferation of the
lymphoid tissue
2 broad classification:
 Hodgkin’s d’se and Non-Hodgkin’s lymphoma

4.  Divided into 2 general prognostic groups:
Indolent lymphomas-relatively good prognosis
Aggressive lymphomas
 NHL may also be characterized as low,
intermediate & high grade.
 Low & intermediate predominate in adults
 90% of childhood NHL are high grade.

5.  Highgrade NHLs comprise of 3 histological
subtypes:
Small non-cleaved cells e.g Burkit,s Lymphoma
Lymphoblastic
Large cell lymphoma

6.  Defn:
 It’s a NHL of the high grade type
 Small non-cleaved cell lymphoma, exclusively of
B-cell origin
 Burkitt lymphoma is named after Denis Parsons
Burkitt, 1958,who mapped its peculiar geographic
distribution across Africa

7. 2 epidemiological types:
Endemic BL - African type
Non-endemic - Sporadic BL
*HIV associated BL*

8.  Endemic BL (African type):
 Distributed btn 15oN & 15oS of the equator
(lymphoma belt)
 The area of highest risk for BL in Africa
(incidence 5 – 15 per 100,000 children)
 Within this belt, there are pockets where the
tumor is extremely rare-in high altitude areas
(no known reason)
 Restricted to those areas with annual rainfall
>50cm & an average temp in the coolest month
of over 15.6oC

9.  BL commonly affects children
 Peak age btn 4-7yrs (6-7yr)
 Uncommon below 1yr of age, and <1% of children
get it below 2yrs.
 Less than 10% of patients are diagnosed after the
age of 15yrs
 M:F =2:1

10.  Noknown cause
1 EBV : Epstein-Barr virus, a member of the family Herpesviridae, which can be isolated from tumor
cells in culture,there
is a strong association btn endemic
BL and EBV. Found in 95% of cases.
2 Malaria: chronic severe falciparum malaria
infn lead to intense host response with
proliferation of the lymphoreticucar system,
particularly of the B-lymphocytes.
3 Chromosomal abnormalities :
t(8;14)-80%, t(8;22)-15%, t(2;8)-5%, this leads to
activation of c-Myc oncogene

11.  In
1979, George Klein postulated a three-stage
pathogenic step required for the dev’t of endemic
BL:
EBV transforms B cells & immortalizes them;
An env’tal factor, e.g holoendemic malaria promotes
polyclonal proliferation of B cells; and
A cytogenetic error emerges & endows the cells with
survival advantage
4 Oncogenes: These are genes which cause
cancer
4 Hiv Infection: In HIV associated burkitts
lymphoma

12.  Endemic BL
 Presents with jaw swelling in 75%
 Maxillae are affected more frequently than the
mandibles
 Maxillary tumor often involves the orbit as well
 The first clinical evidence is often loosening of
teeth
 Non-jaw tumors present mainly as abdominal
mass in ~60%

14.  Virtually any abdominal organ can be involved-
liver, kidneys, ovaries, suprarenal &
retroperitineal LNs, may have Ascites
 CNS involvement- 3rd most common mode of
presentation
 Seen in ~30% of pts
 Often presents as cranial nerve palsy with or
without malignant spinal fluid pleocytosis
 Peripheral node involvement is rare in endemic
cases.

15.  Pleura, Endocrine glands, Testis, skin may be involved
 Bone marrow only 7-8% even after multiple relapses
 Parotid glands

17.  In non-endemic areas;
 The most common site of presentation is with
abdominal d’se in 90% (often ovary & ileocaecal)
 Peripheral node d’se –in 20% of pts
 Jaw involvement -10%
 CNS involvement-5%
 *jaw tumor most common in young children while
abdominal d’se increases in frequency with age*

18.  Jaw: Disfigurement, loosening and loss of teeth,
halitosis, difficulty feeding and speech
 Abdomen: Masses, distention, pain,
constipation, diarrhea, difficulty breathing,
obstructive uropathy, IO, and GI perforation.
 Orbit: Proptosis, altered vision, disfigurement
 CNS/PNS: LOC, cranial nerve palsies, sphincter
abnormalities (retention or incontinence),
paraplegia, etc.
 Fever, loss of appetite, loss of weight, frequent
morbidity

19.  Investigations:
 Aims:
Diagnostic
Staging & pre-chemotherapy
Follow up
o CBC:
Neutrophil (ANC) >1000/ul
Hb >7g/dl
PLT 150,000/ul

20. o Chemistries:
RFT-Creatinine, BUN
Electrolytes-K, PO4-, Ca, Na
LFT
o Lactate dehydrogenase (LDH):- elevated (poor
prognostic factors), correlation with increased
tumor burden
o HIV serology

21. o Imaging studies:
Abdominal US
CXR
CT scan
Echocardiography
o Cytogenetic studies

22. o Procedures:
Biopsy for histology
LP for CSF cytology
BMA or biopsy for staging purpose
 Histology:
 The characteristic histological features of BL is the
so-called “Starry Sky” appearance. imparted by
scattered macrophages with phagocytes cell debris

23.  Staging of BL:
 Stage A : Solitary extra-abdominal site
 Stage AR : Resected intra-abdominal tumor
 Stage B : Multiple extra-abdominal sites
 Stage C : Intra-abdominal tumor with or without
facial tumor
 Stage D : Intra-abdominal tumor with sites other
than facial.

24.  Goal of Rx is cure
 BL can be treated by surgery & chemotherapy
 BL is insensitive to radiotherapy.
 Surgical approaches include:
Biopsy for diagnosis
Debulking-reduction of tumor volume
Laminectomy to relieve spinal cord compression
Insertion of omaya reservoir for intraventricular therapy.

25.  Chemotherapy :
 Isthe Rx of choice
 BL is highly sensitive to chemotherapy.
 Pre-chemotherapy medications:
Fluid preload-orally or IV to ensure high urinary output
Allopurinol 4-5 days before and after administration of
drug
Correction of any metabolic imbalance or haematologic
abnormality
Dexamethasone

26. Divided into high risk and low risk Patient
 Low Risk : Extra-abdominal tumor <10 cm
 High Risk : All, other than above, eg CNS, intra-
abdominal, extra-abdominal tumour >10cm,etc

27.  Combination chemotherapy is used:
 1st line (COM):
• Cyclophosphamide
• Oncovin (Vincristine)
• Methotrexate (MTX)
• + IT MTX + cytocine arabinoside:
Prophylactic x 3 courses(for low risk)
Therapeutic in CNS d’se x all 6 courses(for high risk)
 Course repeated every 2 weeks x 6 courses.

28.  90% curable especially early disease
 Tumour burden (total tumor volume)
◦ clinical stage
◦ serum LDH
 Tumor lysis syndrome
>90% tumor reduction by surgery
 Early relapse (3 month), CR 50% vs. late
relapse, CR 90%.
 CNS relapse in African Burkitt’s, is not a bad
prognostic factor

29. Radiotherapy,
BL is a radio-sensitive tumour but b’se of it’s rapid
growth, radiotherapy is ineffective
Tumour regrows btn @ day’s therapy
Prophylactic craniospinal irradiation achieved no
results in preventing or delaying CNS relapse

31.  ANY QUESTIONS?