This guidance provides a data-supported approach to the diagnosis, risk stratification, treatment and follow up of paediatric patients diagnosed with Burkitt lymphoma.
Level Of recommendation |
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1-Work up for newly diagnosed NHL |
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Pathology specimen is recommended with the proper IHC. |
Strong Recommendation |
We recommend whole body FDG- PET CT if available otherwise contrast enhanced CT neck, chest, abdomen and pelvis is recommended. |
Strong Recommendation |
Bilateral bone marrow aspiration and biopsy is recommended as well as CSF examination. |
Strong Recommendation |
2-Treatment of clinical group A |
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Two 21-day cycles COPAD are recommended. |
Strong Recommendation |
Response assessment is recommended to include imaging studies of primary tumour site. |
Strong Recommendation |
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Multiagent chemotherapy is recommended starting with pre phase COP Followed by response assessment post COP Then administer 2 induction courses COPADM3 and two consolidation courses CYM. |
Strong Recommendation |
Contrast enhanced CT neck, chest, abdomen and pelvis is recommended for response assessment after COP and FDG- PET/CT is recommended for assessment after CYM I. |
Strong Recommendation |
Response assessment after CYM I: · If in CR, then continuation of CYM II is recommended. · If not in CR, biopsy is recommended. If biopsy is not feasible then continue as group B if PET/CT is negative. Upgrade to group C if biopsy is viable or PET/CT is positive. |
Strong Recommendation |
Rituximab addition to chemotherapy is recommended in all high-risk group B |
Strong Recommendation |
4-Treatment of clinical group C |
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Multiagent chemotherapy should be initiated with pre phase (R-COP), followed by 2 induction courses (R-COPADM8), 2 consolidation courses (R-CYVE) and 2 maintenance courses (Sequences 1 and 2) |
Strong Recommendation |
Rituximab addition to chemotherapy is recommended for all group C patients |
Strong Recommendation |
For Group C CNS disease: We recommend a total of 3 intrathecals in pre phase COP and high dose methotrexate after CYVE1 |
Strong Recommendation |
5-End of treatment evaluation |
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End of treatment evaluation should be done and if in CR then follow up is recommended. If not in CR, repeat biopsy from suspicious lesions, and if relapse is confirmed, start relapse protocol. |
Strong Recommendation |
6-Treatment of relapse or refractory disease |
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Combination chemotherapy is recommended with regimen: (R-ICE) Rituximab, ifosfamide, carboplatin, etoposide and intrathecal chemotherapy |
Strong Recommendation
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7- Surveillance (follow up after end of treatment) |
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Routine scans are not recommended unless clinically suspicious. Monthly clinical examination is recommended for the first 3 years then annually. |
Strong Recommendation |