Burkitt 淋巴瘤

Buzzwords → Dx

Diagnostic Criteria

• WHO 5e / ICC 2022: highly aggressive B-cell lymphoma with MYC rearrangement (single-hit), monomorphic medium-sized blasts, near-100 % Ki-67, BCL2-, BCL6+, CD10+ CD20+.
• Distinguish from HGBL with MYC + BCL2/BCL6 — that's a separate WHO entity.
• 3 epidemiologic forms: endemic (African, EBV+, jaw, children), sporadic (Western, ileocecal/abdominal, adolescents/adults), immunodeficiency-associated (HIV/post-transplant).
• CSF + marrow involvement common at diagnosis — staging requires CSF cytology/flow + marrow.

Workup

• STAT (presentation often emergent — TLS, mass effect, CNS).
• CBC, CMP, LDH, uric acid, phosphate, calcium, K baseline + q6h × first cycle.
• Excisional biopsy with full IHC + FISH for MYC, BCL2, BCL6 (rule out HGBL).
• Marrow + CSF analysis (cytology + flow) at diagnosis.
• PET-CT for staging.
• HIV / HBV / HCV / EBV serology.
• Echo / MUGA before anthracycline.
• Pregnancy test in females.

Treatment Algorithm

flowchart TD
  A[Suspected Burkitt] --> B[Pre-phase: rasburicase + IVF<br>+ allopurinol if prolonged TLS risk]
  B --> C[Confirm path + FISH<br>excisional biopsy + marrow + LP]
  C --> D{Risk group}
  D -- low-risk<br>LDH normal, no bulky, completely resected --> E[DA-EPOCH-R × 3 cycles<br>+ IT MTX/Ara-C]
  D -- high-risk<br>or HIV+ or CNS+ --> F[DA-EPOCH-R × 6 cycles<br>+ IT MTX/Ara-C × 6+<br>or CODOX-M/IVAC alternating]
  E --> G[End-of-treatment PET<br>Deauville 1-3 = CR]
  F --> G
  G --> H{Relapse / refractory?}
  H -- yes --> I[Salvage chemo + auto-HCT<br>or CD19 CAR-T (off-label)<br>poor prognosis]

Pearls / Pitfalls

• Spontaneous TLS at presentation is classic — patients can be in florid TLS before any chemo. Rasburicase + aggressive IVF is the right answer; allopurinol alone is insufficient.
• DA-EPOCH-R is preferred for adults (NCI 9177 / lower-intensity than R-Hyper-CVAD/M-IVAC, equivalent efficacy in HIV-associated and sporadic Burkitt).
• CNS prophylaxis is MANDATORY even if CSF is clear — IT MTX/Ara-C × 4–6 doses minimum, often + HD-MTX systemic (Burkitt has 30 % CNS relapse risk without prophylaxis).
• Burkitt vs HGBL with MYC + BCL2: Burkitt is BCL2-negative by IHC and has single-hit MYC. HGBL with MYC + BCL2/BCL6 is a separate aggressive lymphoma (treat as DLBCL with DA-EPOCH-R).
• Endemic Burkitt (African) is EBV-positive in ~95 % cases and presents with jaw masses; sporadic in US/Europe is ileocecal mass.
• R-Hyper-CVAD or CODOX-M/IVAC are alternatives with high toxicity; rarely used now in adults outside clinical trials.
• HIV-associated Burkitt: continue ART throughout chemo; outcomes now equivalent to HIV-negative with DA-EPOCH-R.
• Auto-HCT in CR1 is NOT standard for Burkitt; reserved for refractory/relapsed disease.

Cross-references

• Cytogenetics Atlas — t(8;14) MYC, MYC + BCL2 HGBL
• Drug Regimens — DA-EPOCH-R, CODOX-M/IVAC
• TLS — pre-induction prophylaxis
• DLBCL / HGBL — distinguish double-hit
• CNS Lymphoma — Burkitt CNS+ overlaps

相關題目

• Q-049 — Burkitt — pre-induction TLS prophylaxis
• Q-050 — Burkitt — frontline DA-EPOCH-R indication
• Q-051 — Burkitt vs HGBL — distinguishing by FISH and IHC

Sources