Burkitt Lymphoma Flashcards
What is burkitt lymphoma
It is a non-Hodgkin lymphoma and a malignant tumor of B cell. It is the commonest childhood malignancy and it is non-metastatic.
Name the 3 types of burkitt lymphoma in Nigeria
*Endemic- Nigeria (Africa) Sporadic- *non-endemic Immunodeficiency *associated
Describe the endemic burkitt lymphoma , it’s incidence and it’s doubling time
*Endemic commonly affects the abdomen m o r e
*Incidence- peak age is 5yrs. Ratio of Males to Females is 2:1
*Doubling time is ni 24-48 hrs. It is one of the fastest growing tumors
List the causes of burkitt lymphoma
*EBV,
* chronic malaria,
* malnutrition,
* chromosomal translocation- 8.14, 8.22, 8.2
(Cmy-translocation),
* HIV,
*cytomegalovirus
Describe the clinical features of burkitt lymphoma
*Jaw swelling: it is the commonest presentation and is more common in endemic type; may be unilateral or bilateral; with affection of the maxilla. Swelling can spread to eyes and present with proptosis, altered
vision, and disfiguration.
* Gingival swelling and bleeding
- Halitosis (bad breath)
- Weakness of tooth or loos tooth that easily a g e
- Difficulty in feeding
*- Abdominal swelling- common ni sporadic, seen in older children, rapidly progressing and is painless
- CNS signs like paraplegia, blindness, cranial nerve palsy, sphincter abnormalities
SANCTUARY SITES: for burkitt lymphoma
*testes,
*ovary,
*thyroid,
*breast (affection to these sites is a poor prognostic factor)
Investigations of Burkitt lymphoma
*X-ray: soft tissue swelling, dental anarchy (mal-aligned teeth), loss of lamina dura,, dental abscess, osteolytic bone lesion,
multiple teeth loss
* CSF: pleocytosis (WBC), FBC (pancytopenia), E/U/Cr, Calcium phosphate , Uric acid (tumor
lysis acid), CSF LDH to monitor progress of disease (poor prognosis fi CSF LDH is increased),
* abdominal ultrasound, CT, IVU
(for kidney function)
* Specific: tissue biopsy, histology (starry-sky appearance where the macrophages and histiocytes form the stars while the burkitt cells form the sky),
* bone marrow aspiration,fine needle aspiration for cytology (malignant lymphoid cells)
Staging of burkitt lymphoma
STAGING (is done using the Ziegler’s staging)
*Stage A- single extra abdominal tumor site
*Stage B- multiple extra abdominal tumor site
*Stage AR- intra abdominal tumor 90% resectable
*Stage C- intra abdominal tumor
*Stage D- intra abdominal and extra abdominal tumor site
Treatment of burkitt lymphoma
Tumor is highly chemosensitive
Regimen is executed using Modified Ziegler’s regimen
-cyclophosphamide (side effect: hemorrhagic cystitis)
- Vincristine
- Methotrexate
- Steroids
- Cytosine arabinoside
Treatment continuation
Burkitt’s: treat infections with antibiotics
wound debridement
* If there is abdominal tumor, resection if there is jaw mass, surgery and debulking Affection of spine, decompression of spine
* Malaria prophylaxis can be done using proguanil
Prognosis of burkitt lymphoma
Poor Prognostic Factors: age (<24 yrs, >13 yrs), affection of sanctuary site, male gender, affection of CNS and bone marrow
Good prognostic Factors: Stage A & AR, female gender, jaw swelling
Differentials diagnosis of jaw burkitt lymphoma
A:Rhabdomyosarcoma
b. mumps
c. dentalabscess
d. osteomyelitis of jaw bone
e . t r a u m a
ABDOMINAL differential diagnosis
a. TB
b. nephroblastoma
c. hepatoblastoma d. trauma
e. SCD
Eye differential diagnosis
a. Retinoblastoma
b. Orbital Cellulitis c. Neuroblastoma
d. Rhabdomyosarcoma
Spine differential diagnosis
SPINE
a. Spinal trauma
B. Pott’s disease
C. Complicated SCA