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?