Burkit’s lymphoma, By Dr Opiro Keneth

2,956 views

Published on

Presentation By Dr Opiro Keneth at West Nile Palliative Care Association

Published in: Health & Medicine

Burkit’s lymphoma, By Dr Opiro Keneth

  1. 1.  Presenter: Dr Opiro Keneth, Date: 6th July 2012
  2. 2.  Introduction Definition Epidemiology Aetiology Clinical Features Investigations Staging Treatment Prognosis
  3. 3.  Lymphomas are malignant proliferation of the lymphoid tissue2 broad classification: Hodgkin’s d’se and Non-Hodgkin’s lymphoma
  4. 4.  Divided into 2 general prognostic groups: Indolent lymphomas-relatively good prognosis Aggressive lymphomas NHL may also be characterized as low, intermediate & high grade. Low & intermediate predominate in adults 90% of childhood NHL are high grade.
  5. 5.  Highgrade NHLs comprise of 3 histological subtypes: Small non-cleaved cells e.g Burkit,s Lymphoma Lymphoblastic Large cell lymphoma
  6. 6.  Defn: It’s a NHL of the high grade type Small non-cleaved cell lymphoma, exclusively of B-cell origin Burkitt lymphoma is named after Denis Parsons Burkitt, 1958,who mapped its peculiar geographic distribution across Africa
  7. 7. 2 epidemiological types: Endemic BL - African type Non-endemic - Sporadic BL *HIV associated BL*
  8. 8.  Endemic BL (African type): Distributed btn 15oN & 15oS of the equator (lymphoma belt) The area of highest risk for BL in Africa (incidence 5 – 15 per 100,000 children) Within this belt, there are pockets where the tumor is extremely rare-in high altitude areas (no known reason) Restricted to those areas with annual rainfall >50cm & an average temp in the coolest month of over 15.6oC
  9. 9.  BL commonly affects children Peak age btn 4-7yrs (6-7yr) Uncommon below 1yr of age, and <1% of children get it below 2yrs. Less than 10% of patients are diagnosed after the age of 15yrs M:F =2:1
  10. 10.  Noknown cause1 EBV : Epstein-Barr virus, a member of the family Herpesviridae, which can be isolated from tumor cells in culture,there is a strong association btn endemic BL and EBV. Found in 95% of cases.2 Malaria: chronic severe falciparum malaria infn lead to intense host response with proliferation of the lymphoreticucar system, particularly of the B-lymphocytes.3 Chromosomal abnormalities : t(8;14)-80%, t(8;22)-15%, t(2;8)-5%, this leads to activation of c-Myc oncogene
  11. 11.  In 1979, George Klein postulated a three-stage pathogenic step required for the dev’t of endemic BL: EBV transforms B cells & immortalizes them; An env’tal factor, e.g holoendemic malaria promotes polyclonal proliferation of B cells; and A cytogenetic error emerges & endows the cells with survival advantage4 Oncogenes: These are genes which cause cancer4 Hiv Infection: In HIV associated burkitts lymphoma
  12. 12.  Endemic BL Presents with jaw swelling in 75% Maxillae are affected more frequently than the mandibles Maxillary tumor often involves the orbit as well The first clinical evidence is often loosening of teeth Non-jaw tumors present mainly as abdominal mass in ~60%
  13. 13.  Virtually any abdominal organ can be involved- liver, kidneys, ovaries, suprarenal & retroperitineal LNs, may have Ascites CNS involvement- 3rd most common mode of presentation Seen in ~30% of pts Often presents as cranial nerve palsy with or without malignant spinal fluid pleocytosis Peripheral node involvement is rare in endemic cases.
  14. 14.  Pleura, Endocrine glands, Testis, skin may be involved Bone marrow only 7-8% even after multiple relapses Parotid glands
  15. 15.  In non-endemic areas; The most common site of presentation is with abdominal d’se in 90% (often ovary & ileocaecal) Peripheral node d’se –in 20% of pts Jaw involvement -10% CNS involvement-5% *jaw tumor most common in young children while abdominal d’se increases in frequency with age*
  16. 16.  Jaw: Disfigurement, loosening and loss of teeth, halitosis, difficulty feeding and speech Abdomen: Masses, distention, pain, constipation, diarrhea, difficulty breathing, obstructive uropathy, IO, and GI perforation. Orbit: Proptosis, altered vision, disfigurement CNS/PNS: LOC, cranial nerve palsies, sphincter abnormalities (retention or incontinence), paraplegia, etc. Fever, loss of appetite, loss of weight, frequent morbidity
  17. 17.  Investigations: Aims: Diagnostic Staging & pre-chemotherapy Follow upo CBC: Neutrophil (ANC) >1000/ul Hb >7g/dl PLT 150,000/ul
  18. 18. o Chemistries: RFT-Creatinine, BUN Electrolytes-K, PO4-, Ca, Na LFTo Lactate dehydrogenase (LDH):- elevated (poor prognostic factors), correlation with increased tumor burdeno HIV serology
  19. 19. o Imaging studies: Abdominal US CXR CT scan Echocardiographyo Cytogenetic studies
  20. 20. o Procedures: Biopsy for histology LP for CSF cytology BMA or biopsy for staging purpose Histology: The characteristic histological features of BL is the so-called “Starry Sky” appearance. imparted by scattered macrophages with phagocytes cell debris
  21. 21.  Staging of BL: Stage A : Solitary extra-abdominal site Stage AR : Resected intra-abdominal tumor Stage B : Multiple extra-abdominal sites Stage C : Intra-abdominal tumor with or without facial tumor Stage D : Intra-abdominal tumor with sites other than facial.
  22. 22.  Goal of Rx is cure BL can be treated by surgery & chemotherapy BL is insensitive to radiotherapy. Surgical approaches include: Biopsy for diagnosis Debulking-reduction of tumor volume Laminectomy to relieve spinal cord compression Insertion of omaya reservoir for intraventricular therapy.
  23. 23.  Chemotherapy : Isthe Rx of choice BL is highly sensitive to chemotherapy. Pre-chemotherapy medications: Fluid preload-orally or IV to ensure high urinary output Allopurinol 4-5 days before and after administration of drug Correction of any metabolic imbalance or haematologic abnormality Dexamethasone
  24. 24. Divided into high risk and low risk Patient Low Risk : Extra-abdominal tumor <10 cm High Risk : All, other than above, eg CNS, intra- abdominal, extra-abdominal tumour >10cm,etc
  25. 25.  Combination chemotherapy is used: 1st line (COM): • Cyclophosphamide • Oncovin (Vincristine) • Methotrexate (MTX) • + IT MTX + cytocine arabinoside: Prophylactic x 3 courses(for low risk) Therapeutic in CNS d’se x all 6 courses(for high risk) Course repeated every 2 weeks x 6 courses.
  26. 26.  90% curable especially early disease Tumour burden (total tumor volume) ◦ clinical stage ◦ serum LDH Tumor lysis syndrome >90% tumor reduction by surgery Early relapse (3 month), CR 50% vs. late relapse, CR 90%. CNS relapse in African Burkitt’s, is not a bad prognostic factor
  27. 27. Radiotherapy,BL is a radio-sensitive tumour but b’se of it’s rapid growth, radiotherapy is ineffectiveTumour regrows btn @ day’s therapyProphylactic craniospinal irradiation achieved no results in preventing or delaying CNS relapse
  28. 28.  ANY QUESTIONS?

×