| Description |
Burkitt's Lymphoma exists in an endemic form in equatorial Africa and
a sporadic form throughout the rest of the world. The endemic form
often presents as a mandibular mass and has a predilection for the abdominal
viscera. The sporadic form usually invoves the ileocecum and peritoneum.
The African form is associated with EBV infection. The malignant
cells are mature B lymphocytes. Morphologically, the field look like
a "starry sky" because of the presence of macrophages with tingible substance
(the remains of apoptotic cells). The cells have a high nuclear to
cytoplasmic ratio. There are lipid vacuoles in the cytoplasm, and
multiple nucleoli in the nucleus. Burkitts is aggressive but curable. |
| Frequency |
Burkitts accounts for 30% of childhood NHL in the US. The sporadic
form is three times more common in males than females. |
| Cytogenetics |
| Locus |
Product |
Description |
t(2;8)
t(8;14)
t(8;22) |
Rearrangements of myc on chromosome 8. |
The classic t(8;14) involves fusion of
the Ig heavy chain and myc. Myc regulates progression from G1 to
S phase. Different myc breakpoints occur in the various geographic
variants. |
|
| Markers |
| Positive |
sIgM, lambda or kappa chains, B antigens (CD 10, 19, 20, 22, 79a) |
| +/- |
|
| Negative |
CD 5, 23, TdT |
|