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BURKITT’S LYMPHOMA

BURKITT’S LYMPHOMA. Burkitt’s Lymphoma (BL) is a B-cell lymphoma. BL was discovered by Dennis Burkitt, a British Surgeon who was working in Uganda. It is the commonest childhood malignancy in Africa. Also found in South America, Papua New Guinea and very rarely in UK & US.

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BURKITT’S LYMPHOMA

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  1. BURKITT’S LYMPHOMA TA OGUNLESI (FWACP)

  2. Burkitt’s Lymphoma (BL) is a B-cell lymphoma. • BL was discovered by Dennis Burkitt, a British Surgeon who was working in Uganda. • It is the commonest childhood malignancy in Africa. • Also found in South America, Papua New Guinea and very rarely in UK & US. • That is contrary to the pattern in the developed world where Leukaemias are the commonest childhood cancers. TA OGUNLESI (FWACP)

  3. EPIDEMIOLOGY OF BL • Sex: Commoner among males. M:F = 2:1 • Age: 4 to 8 years but peaks at 7 years (Hardly seen < 2yrs) • There are 2 types: Endemic & Sporadic • Endemic type occurs in a defined part of Africa & SE Asia: 10O-16O North & South of the Equator; Temperature > 16OC & Rainfall > 75cm TA OGUNLESI (FWACP)

  4. EPIDEMIOLOGY OF BL • This area corresponds with an area of “malaria holoendemicity” in Africa. • In Nigeria, BL is prevalent in the West & East where humidity & temperature are high but NOT in the North. • Sporadic type occurs in the western world. TA OGUNLESI (FWACP)

  5. AETIOLOGY OF BL • BL is a neoplasm of B lymphocytes. • 3 issues involved in the current concept of African BL: • Early Epstein Barr Virus Infection • Intense and chronic plasmodium falciparum infestation • Chromosomal translocation TA OGUNLESI (FWACP)

  6. AETIOLOGY OF BL • Early EBV infection causes massive proliferation of B-cells because these cells have C3-d –like receptors for the virus. • Anti-EBV antibodies are commonly elevated in pre-BL children. • P. falciparum promotes further enhancement of B-cell proliferation by suppressing T-cells. • T-cells are needed to eliminate EBV-infected cells. TA OGUNLESI (FWACP)

  7. AETIOLOGY OF BL • Chromosome 8 contains C-myc gene, an oncogene which regulates cellular proliferation. • When the C-myc gene is translocated to Chromosomes 2, 14 or 22, it loses its capacity to regulate cellular proliferation. • The final result is uncontrolled excessive proliferation of B-cells. TA OGUNLESI (FWACP)

  8. CLINICAL PRESENTATION • GENERAL SYMPTOMATOLOGY • Weight loss • Anorexia • Recurrent Fever • Malaise TA OGUNLESI (FWACP)

  9. CLINICAL PRESENTATION • Incidence of BL in Africa is 1/10,000 • The incidence in Nigeria used to be 55% at UCH, Ibadan Cancer Registry but more recent reports suggest a decline in incidence to 19%. • The incidence of the other childhood tumors may be on the increase but BL remains the commonest. TA OGUNLESI (FWACP)

  10. CLINICAL PRESENTATION • It is the fastest growing tumor known in man. • Doubling rate is ≈ 24 hours (cell loss rate = 70% of cell renewal rate) • The interval between onset of symptoms & presentation is usually short: Facial tumor presents within an average of 4 weeks while abdominal tumor takes longer. TA OGUNLESI (FWACP)

  11. CLINICAL PRESENTATION • BL commonly affects the face, abdomen and CNS. • In the endemic form of BL, facial bones (Maxilla, mandible and orbits) are mostly affected. • BL of jaw bone is more common in the younger age group (with the peak at 5 yrs). • The abdomen is mostly affected in the sporadic form of BL. It is also commoner in the relatively older population (with the peak at 7 yrs) TA OGUNLESI (FWACP)

  12. CLINICAL PRESENTATION • Kidneys & ovaries are the commonest abdominal viscera affected by BL. • Liver, spleen and lymph nodes are very rarely involved. • CNS involvement may occur either as primary lesion or as relapses. • Facial involvement may be associated with tumors at other sites especially abdomen TA OGUNLESI (FWACP)

  13. CLINICAL PRESENTATION • FACIAL (50% of cases in Ibadan) • Swelling of the affected jaw • Loosening of the teeth • Proptosis • Bleeding & ulceration from the oral cavity • Rapidly growing and painless cervical/submandicular lymph nodes TA OGUNLESI (FWACP)

  14. JAW BL TA OGUNLESI (FWACP)

  15. CLINICAL PRESENTATION • ABDOMINAL (75% of cases in Ibadan) • Abdominal distension • Palpable, craggy, non-tender masses over the affected areas • Ascites • Pressure symptoms like intestinal obstruction • May present with acute renal failure TA OGUNLESI (FWACP)

  16. CLINICAL PRESENTATION • CNS (15% of cases in Ibadan) • Peak age is 9 years • Single or Multiple Cranial nerve deficits: squints, blindness • Paraplegia (spastic or flaccid) • Bladder & Bowel dysfunction • Neck Stiffness, drowsiness & coma TA OGUNLESI (FWACP)

  17. CLINICAL PRESENTATION • No tissue is exempt from tumor involvement • BL does not metastasize but may be multifocal in origin • Involvement of the Reticulo-endothelial system (peripheral lymph nodes and bone marrow) is commoner in the Sporadic Type but very rare in the Endemic type. • It may take the form of: • Reactive lymphopoiesis with peripheral lymphocytosis • Leukaemia TA OGUNLESI (FWACP)

  18. INVESTIGATIONS • HISTOLOGY: That is the hallmark of diagnosis • Histology must be done before chemotherapy • Finding: • Closely packed hyperchromatic monomorphic lymphoid cells interspersed with phagocytic histiocytes. • This is described as STARRY SKY appearance (STARS: Histiocytes; SKY: TUMOR CELLS TA OGUNLESI (FWACP)

  19. TA OGUNLESI (FWACP)

  20. INVESTIGATIONS • Starry Sky appearance is NOT pathognomonic of BL. • May also occur in: • Lymphocytic lymphoma • Hodgkins paragranuloma • Stem cell lymphoma TA OGUNLESI (FWACP)

  21. INVESTIGATIONS • CYTOLOGY • Done on CSF, Ascitic fluid & Fine Needle Tumor Aspirate using any of these method: • Phase Contrast Microscopy (examination of tumor cells in the living state) • Air Dried Smear (examination of dead & fixed cells) • Tumor cells are ROUND CELLS with SCANTY intensely BASOPHILIC CYTOPLASM with large VACUOLES and PROMINENT NUCLEI & NUCLEOLI. TA OGUNLESI (FWACP)

  22. TA OGUNLESI (FWACP)

  23. INVESTIGATIONS • RADIOLOGY • Early bone features include osteolytic lesions manifesting as LOSS OF DENTAL LAMINA DURA (affecting both erupted & unerupted teeth) • These are seen even in the absence of clinically obvious jaw mass • Abdominal Ultrasonographic Scan • Computerized Tomographic Scan • Gallium-67 Scintigraphy TA OGUNLESI (FWACP)

  24. INVESTIGATIONS • Others as necessary include: • CSF analysis: Lymphocytosis and ↓Glucose • Serum Electrolytes, Urea & Creatinine • Serum Uric acid, Calcium & Phosphate • Serum Lactate Dehydrogenase (LDH) – used as a tumor marker to monitor response to treatment & relapse TA OGUNLESI (FWACP)

  25. CLINICAL STAGING • Several staging methods but most popular is the Ziegler & Magrath (1974) A: Solitary extra-abdominal site B: Multiple extra-abdominal site C: Intra-abdominal ± Facial tumor D: Intra-abdominal ± other sites apart from facial AR: Resected (90%) intra-abdominal tumor TA OGUNLESI (FWACP)

  26. DIFFERENTIAL DIAGNOSIS • Abdominal Mass • Neuroblastoma • Nephroblastoma • Abdominal Tuberculosis • Orbital Mass • Retinoblastoma • Neuroblastoma • Acute Lymphoblastic Leukaemia TA OGUNLESI (FWACP)

  27. DIFFERENTIAL DIAGNOSIS • Jaw Mass • Cellulitis • Rhabdomyosarcoma • Dental abscess • Dentigenous cyst • Mandibular osteomyelitis • Ossifying Fibroma • Ameloblastoma TA OGUNLESI (FWACP)

  28. TREATMENT • CHEMOTHERAPY • Drugs: • IV Cyclophosphamide 1000mg/m2 DAY 1 • IV Vincristine 1.5mg/m2 DAY 1 • IV Methotrexate 37.5mg/m2 DAY 1 • Oral Prednisolone 40mg/m2 DAYS 1 to 5 • CNS PROPHYLAXIS • IT Methotrexate 12.5mg/m2 DAYS 1 & 5 OR • Cytosine Arabinoside 30mg/m2 DAYS 1 & 5 TA OGUNLESI (FWACP)

  29. TREATMENT • The drugs are given in a course. • The courses are repeated every 2 weeks • Between 4 and 6 courses are adequate. • Combination therapy increases the long-term survival rate from 20% to 50% TA OGUNLESI (FWACP)

  30. TREATMENT • SURGERY – Only useful in advanced BL. Mortality can be reduced significantly if surgical resection of at least 80 – 90% tumor bulk is done before chemotherapy • RADIATION – Less useful because of toxicity. • IMMUNOAUGMENTATION - New • BONE MARROW TRANSPLANTATION – New, innovative & experimental TA OGUNLESI (FWACP)

  31. PROGNOSIS • Good • Isolated jaw mass • Completely resectable mass • Bad • Male sex • Intra-abdominal mass • Relapse • CNS involvement • Bone marrow involvement TA OGUNLESI (FWACP)

  32. ACUTE TUMOR LYSIS SYNDROME • Acute metabolic emergency in childhood cancers • It occurs in rapidly growing solid tumors and leukaemias. • It is caused by massive destruction of cancer cells either: • Spontaneously due to inadequate blood supply to the tumor • Following massive cellular destruction by drugs TA OGUNLESI (FWACP)

  33. ACUTE TUMOR LYSIS SYNDROME • The cellular metabolites, particularly, uric acid are released into the circulation • The renal excretory system may be overwhelmed by the load of uric acid. • This causes renal tubular damage resulting in renal failure and several systemic complications TA OGUNLESI (FWACP)

  34. CARDINAL FEATURES OF ATLS • Hyperuricaemia • Hyperphosphataemia • Hyperkalaemia • Hypocalcaemia • Metabolic acidosis • Hypoglycaemia TA OGUNLESI (FWACP)

  35. PRESENTATION OF ATLS • Tetany • Positive Chvostek’s sign • Cardiac arrhythmias • Diaphoresis • Seizures • Hyperventilation • ECG changes (peaked P wave, elevated ST segment) • Anuria • Fluid retention (Hypertension, CCF) TA OGUNLESI (FWACP)

  36. PREVENTION OF ATLS • Pre-chemotherapy allopurinol (a Xanthine Oxidase inhibitor) 10mg/kg/day for 48 – 72 hours before commencement of cytotoxics • Hyperhydration (3Litres/m2 of 5% Dextrose Saline infusion per day) • Diuretics (IV Frusemide 2mg/kg) TA OGUNLESI (FWACP)

  37. PREVENTION OF ATLS • Uricaise (500 – 1000i.u daily) if serum uric acid remains elevated despite allopurinol use. • Alkanization of urine with Sodium Bicarbonate infusion. • Specific treatment of electrolyte derangements • Dialysis is best used when ATLS is established TA OGUNLESI (FWACP)

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