BURKITS LYMPHOMA
Definition
It is a high-grade B-cell neoplasm and has 2 major forms, the
endemic (African) form and the nonendemic (sporadic) form.
Burkitt lymphoma is a childhood tumor but it is observed in
adult patients.
Burkitt lymphoma is one of the fastest growing malignancies
in humans, with a very high growth fraction.
Pathophysiology:
Burkitt lymphoma is a monoclonal proliferation of B
lymphocytes characterized by small noncleaved cells that are
uniform in appearance and that produce a diffuse pattern of
tissue involvement.
-Under the microscope, Burkitt lymphoma is characterized
by the presence of a "starry sky" appearance (also observed
in other highly proliferative lymphomas), imparted by
scattered macrophages with phagocytes cell debris.
-The African form most often involves the maxilla or
mandible. The involvement of abdominal organs, such as the
kidneys, ovaries, or retroperitoneal structures, is slightly less
common. In contrast, the sporadic form usually involves
abdominal organs, with the most common involvement of the
distal ileum, cecum, or mesentery and less common
involvement of other abdominal organs, pelvic organs, and
facial bones.
Most Burkitt lymphomas carry a translocation of the c-myc
oncogene from chromosome 8 to either the immunoglobulin
(Ig) heavy-chain region on chromosome 14 [t(8;14)] or one
of the light-chain loci on chromosome 2 (kappa light chain)
[t(8;2)] or chromosome 22 (lambda light chain) [t(8;22)].
The Epstein-Barr virus (EBV) has been implicated strongly
in the African form, while the relationship is less clear in the
sporadic form.
-EBV is associated with about 20% of sporadic cases.
-Rare adult cases are associated with immunodeficiency,
particularly AIDS.
-The lymphocytes have receptors for EBV and are its specific
target.
-In the African form, the hosts are believed to be unable to
mount an appropriate immune response to primary EBV
infection, possibly because of coexistent malaria or another
infection that is immunosuppressive. Months to years later,
excessive B cell proliferation occurs
Epidemiology
Burkitt lymphoma is endemic in certain regions of
equatorial Africa and other tropical locations between
latitudes 10° south and 10° north. Incidence in these
areas of endemic disease is 100 per million children.
Mortality/Morbidity:
With combination chemotherapy and CNS
prophylaxis, the survival rate is now at least 60%.
Patients with limited disease have a survival rate of
90%.
Patients with bone marrow and CNS involvement
have a poor prognosis. Adults with the disease,
especially those in the advanced stage, do more
poorly than affected children.
Sex:
The male-to-female ratio is 2-3:1.
Age:
-Burkitt lymphoma is most common in children. In
Africa, the mean age is 7 years.
-Outside Africa, the mean age is 11 years.
Clinical presentation
History:
In the African form
 Most often present with swelling of the
affected jaw other facial bones, loosening of
the teeth
 Swelling of the lymph nodes, which are
nontender and rapidly growing, in the neck or
below the jaw.
 Abdominal presentation is slightly less
common
 Wt loss, Night sweat, generalized fatigue.
Sporadic form
 Abdominal tumors causing swelling and pain
in the affected area.
 Some patients present with symptoms of
bowel obstruction secondary to an ileal-cecal
intussusception caused by tumor growth.
-Because of the rapid growth of the Burkitt tumor,
patients may quickly manifest significant metabolic
derangement and renal function impairment
)The most commonly used staging system for NHL
 in children is that of the St. Jude's Children's
-Less common presentations of Burkitt lymphoma include an Research Hospital.
epidural mass, skin nodules, CNS symptoms, and bone (Stage I)
marrow involvement. It separates patients with a single tumor or diseased
-Rare cases of Burkitt lymphoma can present as acute lymph node
leukemia (L3-ALL) with fever, anemia, bleeding, and (Stage II)
adenopathy. or two or more tumors or diseased lymph nodes on
Physical: the same side of the diaphragm
Major signs of Burkitt lymphoma include a soft tissue mass (Stage III)
associated with the involvement of the jaw or other facial from those with a large chest or abdominal tumor or
bones, enlarged cervical lymph nodes, abdominal masses, (Stage IV)
and ascites. Involvement of the bone marrow and central nervous
system
Staging
The most common system of staging for non-Hodgkin's Investigations
lymphomas in adults, including Burkitt's lymphoma, is the Lab Studies:
Ann Arbor system. 1.FBC and ESR
Stage I: 2.Electrolytes-serum levels of sodium, potassium,
- Limited to one group of lymph nodes either above or calcium, phosphorus, magnesium
below the diaphragm, 3.Urea, and creatinine
- Organ or part of the body other than the lymph nodes, 4.Uric acid levels
but has not spread to other organs or lymph nodes. 5.Liver function tests.
Stage II: 6.Biopsy of suspected lymph nodes or other disease
- The lymphoma in two or more lymph node groups on sites, including bone marrow, is essential for the
the same side of the diaphragm diagnosis.
- only one organ involved and has spread to the lymph 7.FNA of the lymph node may also be done.
nodes near that organ 8.Cerebrospinal fluid (CSF) also should be evaluated
Stage III: in all cases.
- The lymphoma is present in groups of lymph nodes 8.Cytogenetic studies of peritoneal or pleural fluid,
on both sides of the diaphragm. when appropriate, should be performed for diagnostic
- It may involve an organ or site outside the lymph or staging purposes.
nodes, the spleen, or both. Imaging Studies:
Stage IV: 1. CXR -rule out lung metastasis and mediastinal
- The lymphoma is disseminated (spread) throughout involvement. Chest CT should be performed if chest
one or more organs outside the lymph nodes. There x-ray is abnormal
may or may not be involvement of lymph nodes that 2.Abdominal U/S
are remote from the affected organs. 3. Abdominal CT scan -retroperitoneal and
For each of stages mesenteric lymph nodes, liver, kidneys, ovaries, and
A -No general symptoms other structures.
B- Is used for patients with any of the following: 4.Head or spinal CT scan or MRI is indicated if
 Unexplained loss of more than 10% of body weight in neurological signs and symptoms are present.
the last six months 5.Bone scan and plain bone radiographs are needed
 Unexplained fever for patients with symptoms of bone involvement.
 Drenching night sweats
E - malignancies outside the lymph nodes
Other sites in the body are identified with additional letters,
such as D for the skin or H for the liver.

Histologic Findings: Lymph node involvement occurs.

Burkitt cells are homogeneous in size and shape, with round
to oval nuclei and slightly coarse chromatin, with multiple
nucleoli, and with intensely basophilic vacuolated cytoplasm
that contains neutral fat. Frequent mitotic figures usually are
observed. A starry sky appearance is imparted by scattered
macrophages with phagocyte cell debris.