Burkitt Lymphoma

BURKITT’S
LYMPHOMA
OUTLINE
■ INTRODUCTION
■ VARIANTS
■ PATHPHYSIOLOGY
■ CLINICAL FEATURES
■ CLASSIFICATION AND DIAGNOSIS
■ TREATMENT
■ PROGNOSIS
Introduction
■ Lymphomas are malignancies of the
lymphoid system, hence may arise at any
site where lymphoid tissue is present
■ Divided broadly into hodgkin and non
hodgkins depending on the presence of
reed-sternberg cell.
■ Burkitt lymphoma is a form of non
hodgkin and is a malignant tumour of B
cell origin
■ fastest growing tumour in man with
doubling time of 8-24 hrs
■ It was described by Burkitt in 1958
as a malignant process involving
typically maxilla and mandible or
abdominal viscera
Variants of Burkitt’s lymphoma
■ There are 3 variants of BL

■ Endemic BL
■ Sporadic BL
■ Immune deficiency associated BL
Endemic Burkitt’s
■ It is the commonest childhood
tumour in the tropical region
■ About 60-65% in our centre
■ 80% of all NHL
■ Peak incidence 4 -7 years
■ M>F ratio 1.7-2:1
■ Involves extranodal sites
Burkitt belt
■ Corresponds with intense malaria infestation
■ 10- 15 degrees N and S of the equator
■ Altitude ≤ 1500m
■ Annual rainfall ≥ 50cm
■ Ambient temperature ≥26.6 degrees celsius
Burkitt Belt img
Sporadic Burkitt’s
■ Western world
■ Low incidence: 1-2% of all lymphomas
■ Affects both children and adults, poor
prognosis in adults
■ Median age 30
■ M>F =3 :1
■ Abdominal masses common majorly ileocecal
region affected
Immune deficiency associated
■ This variant of Burkitt lymphoma is most
common in people with HIV/AIDS
■ accounts for 30% to 40% of non-Hodgkin
lymphoma in HIV patients and may be an
AIDS-defining disease
■ Can also occur in people with congenital
conditions that cause immune deficiency
and in organ transplant patients who take
immunosuppressive drugs
Pathophysiology
■ EBV infection
■ P.falciparum infection
■ Chromosomal Translocation
Pathophysiology
■ EBV infection stimulate B cell
proliferation
■ P.falciparum infection suppresses CD 4
and CD 8 T cells that normally destroy
abnormal cells
■ Translocations involving the C-myc gene
on Chr 8q causes loss of the c-myc
gene function so that proliferation
occurs unchecked
Pathophysiology
■ Malignant transformation of EBV

infected cell occur when there is
genetic translocation of C-MYC which
result into burkitt lymphoma
EBV and the Burkitt’s
lymphoma variants
■ Endemic– 30% +ve for EBV
■ Sporadic-- 10 to 30% +ve for EBV
■ Immune deficiency associated– 25 to 40%
+ve for EBV
Clinical features
■ Usually present as a painless hard craggy
mass which could either be
– Abdominal
– Extra abdominal which include jaw,
CNS and other sites
Presentation is as early as 2weeks in jaw

mass and 4weeks in case of abdominal
mass
Clinical features
■ Jaw burkitt is more common in the endemic

type usually in younger age group
■ The commonest extra abdominal tumour
■ Affects maxilla more than the mandible and
causes
– dental anarchy
– loosening of teeth
– gingival swelling
■ It also affect the orbit and cause proptosis
Clinical Features
■ Abdominal burkitt is the commonest

presentation in the non endemic area
■ affects the mesenteric and the
retroperitoneal nodes
■ Can also affect kidney, liver, spleen and
ovaries
Clinical Features
■ CNS burkitt is almost always with jaw
or abdominal mass and it causes the
following
– cranial nerves palsy
(2,3,4,5,6,7,rarely10)
– flaccid paraplegia due to vascular
compromise and cord compression
– CSF pleocytosis
– altered consciousness
– Raised ICP
Clinical features
■ Other sites that are rarely involved are
– thyroid gland
– Testes
– Bone marrow
– salivary gland
– Lung
■ Tumour lysis syndrome; a derangement in
biochemical parameters in patients with tumors
– it can be spontaneous but commonly follow
chemotheraphy related tumour necrosis
■ Other symptoms associated with Burkitt
lymphoma include:
■ Loss of appetite
■ Weight loss
■ Fatigue
■ Night sweats
■ Unexplained fever
Investigations
■ Supportive
■ Supportive
Supportive
■ FBC +platelets -- pancytopenia indicates BM
invasion
■ EUCr -- to monitor metabolic complications
■ CSF LDH -- for prognosis
■ Xray for jaw lesions
■ Abd USS/CT scan—to r/o Abdominl
involvement
■ LP for CSF cytology — to r/o CNS involvement
■ BM aspiration for staging
X ray findings
■ Osteolytic lesion
■ loss of lamina dura-- characteristic of
jaw involvement even in the absence of
swelling
■ Dental anarchy(malalignment)
Specific
■ Biopsy/FNAC- Burkitt’s cell
■ Histology- starry sky appearance
■ Immunophenotyping
■ Genetic studies
Biopsy/FNAC
■ Burkitt Cell-
– Small to medium sized
– Round or oval monomorphic cells
– Scanty cytoplasm that is intensely
basophilic
– Multiple vacuoles in the cytoplasm
– Large nucleus with multiple nucleoli
Burkitt cells, Wright stain
Immunophenotyping
■ The use of certain dyes to determine
the CD on malignancy cells
■ CD 10, 19, 22, 79a and Pax-5 are pan B
cell antigens
■ KI67 /MIB-rate of division
■ 80-100% in Burkitt’s (very high in
rapidly dividing cells)
■ BCL 6 (germinal centre marker) and
BCL 2(apoptotic marker) are negative in
Burkitt’s
Histology
■ Starry sky appearance
■ The tumor cells are closely apposed to
each other, forming a dark blue
background (the “sky”)
■ Tingible body macrophages that have
ingested the cell debris (the “stars”)
Starry sky appearance
Genetic studies
1. The most common variant is t(8;14)
which accounts for about 95 %of
cases. This involves c-myc and IGH
2. t(8;22) involving IGL and c-myc
– IGL@ contains genes for the
lambda light chains of Ig
3. T(2;8) involving IGK@ and c-myc
– IGK@ contains genes for the kappa
(κ) light chains of Ig
Staging-Ziegler staging
■ A-single extra abdominal site
■ B-multiple extra abdominal site (excluding
BM & CNS)
■ AR-intra-abdominal tumour >90% resectable
■ C - abdominal involvement
■ D-CNS or BM involvement)
Differential diagnosis
■ For jaw burkitt

– dental cyst(dentrigenous cyst)- hard
– Osteomyelitis of the jaw- pinpoint
tenderness
– Rhabdoyosarcoma- slow growing and
hard
– Histiocytic lymphoma- slow growing
For abdominal burkitt
■ Neuroblastoma- 1-2yrs of age
■ Nephroblastoma – 1-2yrs of age, cause is
embryonic
■ Abdominal Tb- not as fast growing
■ Ovarian cyst
■ Retroperitoneal rhabdomyosarcoma- difficult to
palpate
For CNS
■ TB of the spine
– Causes spastic paraplegia while
burkitt causes flaccid paraplegia
■ Acute leukemia
■ Other intradural tumours
For orbital involvement
■ Metastatic neuroblastoma- grows on bony
surface and pushes the globe out and may
have bumps on their head which are
painless
■ Chloroma- Eye deposition of AML
■ Retinoblastoma- embryonic tumour and
occurs at a very young age
Treatment
■ Treatment regime include supportive
and definitive therapy
■ DEFINITIVE THERAPHY; is a cancer
chemotherapy with the use of cytotoxic
drug
■ The agents may be used as single
agent or in combination with other
drugs (to employ the synergistic
property of each drugs and reduce the
rate of tumour resistance )
■ BL is a very chemosensitive tumour,80-
90% will remit with single course of
treatment
■ Usually 4 courses of treatment are given
but for CNS BL and relapse, 6 course of
treatment are used
Precaution before chemotherapy
■ FBC before chemotherapy at least

– WBC of 2000/mm3
– Absolute neutrophil count of 1000/mm3
– PCV at least 30%
– Platelet of 100000/mm3
Treatment regimens
■ 1st line drugs ■ For CNS burkitt 3 drugs
are used
– Cyclophosphamide
– Methotrexate
– Oncovin(vincristine)
– Hydrocortisone
– Metotrexate
– Cytosine
■ 2nd line drugs arabinoside
– Ifosfamide
– Etoposide
– Cytarabine
■ Surgery use only for debulking
■ Radiotheraphy not used in BL because the
dose required to cure the tumour is toxic to
other cells in the body
■ Immunotheraphy use of vaccines to
stimulate the host’s immune system to
reject cancer cells
Complication of cytotoxic drugs
■ General side effects
■ Cytotoxic drugs affect other rapidly dividing
cells in the body as well as the tumor cells
– bone marrow-myelosuppression
– gut-nausea,vomiting and diarrhoea
– lymphoid tissues-hyperproliferation
– hair follicles-alopecia
– foetus-teratogenicity,abortion
– Spermatozoa-sterility
Specific Side effects
■ Cyclophosphamide- haemorhagic
cystitis(due to accumulation of acrolein in
the bladder) prevented by administration of
mesna
■ Cytarabine-megaloblastic anaemia,cerebral
damage and hepatotoxicity
■ Methotrexate-mucositis,haemorhagic
enteritis, diarrhoea and cerebral atrophy
■ Hydroxyurea-maculopapular rash,impaired
renal function,stomatitis and megaloblastic
aneamia
Tumor lysis syndrome
■ Derangement in biochemical parameters
due to massive cell lysis
■ Hyperphosphatemia
■ Hyperkalemia
■ Hyperuricemia
■ Hypocalcemia
■ Hypoglycemia
■ Metabolic acidosis
Supportive Treatment
■ Adequate hydration to prevent
crystallization of uric acid (2-3L/m2)
■ Allopurinol 24-48hrs 100mg tds before
starting chemotherapy and continue
through out the course of therapy
■ Bicarbonate infusion to alkalinize urine
to correct acidosis
■ Manage side effects
■ Malaria prophylaxis ( paludrine) to
prevent malaria
■ Antibiotics
■ Analgesia
■ Adequate bed rest
■ Relapse means reoccurrence of the tumour
after remission
■ Within 3month of remission—Early relapse
■ More than 3month– Late relapse
■ Primary-- Relapse at old site
■ Secondary-- at a new site
PROGNOSIS
■ BAD ■ GOOD
1. Ziegler stage C and D 1. Relapse at old site
2. Involvement of sanctuary 2. Ziegler stage A and B
sites- testes, thyroid and 3. Low serum LDH
bone marrow
3. Early relapse at new site
4. Immunodeficieny
5. Age less than 2 and
greater than 9
■ tumour relapse is common
■ 40-80% have 2years survival in some report
■ Mortality rate is 65%.
THANK YOU

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BURKITT’S

■ There are 3 variants of BL

■ Malignant transformation of EBV

Presentation is as early as 2weeks in jaw

■ Jaw burkitt is more common in the endemic

■ Abdominal burkitt is the commonest

■ For jaw burkitt

■ FBC before chemotherapy at least

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