何杰金氏淋巴瘤

Buzzwords → Dx

Diagnostic Criteria

• Excisional biopsy is mandatory (FNA insufficient for architecture).
• Classical HL: CD30+ CD15+ CD20-/+ PAX5-dim with Reed-Sternberg / Hodgkin cells.
• Nodular lymphocyte-predominant B-cell lymphoma (NLPBL): CD20+ CD79a+ BCL6+ OCT2+ BOB1+ CD30- CD15- ("popcorn"/"L&H" cells in nodular pattern). Renamed in WHO 5e/ICC 2022 — no longer "Hodgkin".
• Staging: Lugano (Cotswold-modified Ann Arbor) I–IV with A/B suffix and X (bulky), E (extranodal contiguous).

Workup

• PET-CT (staging + PET-2 interim assessment + end-of-treatment Deauville score).
• Excisional biopsy with full IHC panel.
• CBC + CMP + LDH + ESR + albumin + β2-microglobulin + HIV/HBV/HCV.
• HIV testing (epidemiologic association with MC-cHL).
• Echo / MUGA before anthracycline; PFTs before bleomycin.
• HSV/VZV serology (immunosuppression).
• Fertility counseling (chemo + RT — alkylators in BEACOPP).

Treatment Algorithm

flowchart TD
  A[Confirmed cHL] --> B{Stage}
  B -- Stage I-II<br>favorable --> C[ABVD × 2 + ISRT<br>or ABVD × 4 alone if PET-2 negative<br>RAPID/EORTC H10]
  B -- Stage I-II<br>unfavorable --> D[ABVD × 4 ± ISRT<br>or escalated BEACOPP × 2 + ABVD if PET-2+]
  B -- Stage III-IV --> E[BV-AVD × 6<br>S1826 - new standard]
  E --> F{PET-2 response}
  C --> F
  D --> F
  F -- Deauville 1-3 = CR --> G[Complete planned chemo]
  F -- Deauville 4-5 --> H[Switch to escalated BEACOPP<br>or BV-AVD intensification]
  G --> I{R/R disease?}
  I -- yes --> J[Salvage: BV + nivolumab/pembrolizumab<br>then auto-SCT]
  J --> K[Post-auto BV maintenance<br>AETHERA in high-risk]
  I --> L{Multiply relapsed post-auto?}
  L -- yes --> M[Allo-HCT or<br>nivolumab/pembrolizumab indefinite]

Pearls / Pitfalls

• Bleomycin pulmonary toxicity — risk factors: age >40, smoking, renal impairment, cumulative dose >270 U, oxygen therapy. Modern strategies omit bleomycin after PET-2- (RATHL trial). G-CSF + bleomycin = synergistic pneumonitis — avoid concurrent unless absolutely necessary.³
• BV-AVD (SWOG S1826) is now standard for advanced HL, beating BV-AVD vs ABVD with better PFS and lower neutropenic fever vs nivolumab + AVD even (data evolving). For age ≥60 — fewer pulmonary AEs vs ABVD.²
• PET-2-adapted therapy: Deauville 1-3 = continue planned, drop bleomycin in advanced (RATHL); Deauville 4-5 = escalate (BEACOPP or BV-AVD).
• NLPBL ≠ classical HL. ICC 2022 renamed NLPHL to NLPBL (it's B-cell). Treat early-stage with rituximab ± RT or watch-and-wait; advanced with R-CHOP / R-CVP / BR.
• Brentuximab vedotin (anti-CD30 ADC) → peripheral neuropathy (cumulative); avoid concurrent bleomycin (pulmonary toxicity), monitor NCS.
• Checkpoint inhibitors in HL (nivolumab, pembrolizumab) → highly active (~70 % ORR) in R/R cHL; can serve as bridge to allo-HCT. Watch for irAEs (especially in pre-allo setting → hyperacute GVHD).
• Lhermitte sign post-mediastinal RT — transient electric shock with neck flexion, due to demyelination; usually resolves.
• Late effects: secondary cancers (NHL, breast/lung/thyroid), CV disease (RT to mediastinum + anthracycline), hypothyroidism, infertility (more with alkylators).
• AETHERA trial: post-auto BV maintenance for high-risk R/R cHL (refractory, <12-mo CR1, B-symptoms at relapse, extranodal at relapse) → improves PFS.
• AYA + early-stage HL: preserve fertility (sperm banking, ovarian protection); choose lowest-toxicity effective regimen.

Cross-references

• Staging — Lugano, IPS for advanced HL
• Drug Regimens — ABVD, BV-AVD, BEACOPP
• DLBCL — contrast aggressive NHL
• Auto-HCT — relapsed cHL
• Pain — bone pain post BV

相關題目

• Q-035 — HL — frontline regimen for advanced-stage classical HL (S1826 era)
• Q-036 — HL — bleomycin + G-CSF combination
• Q-037 — HL — NLPBL (formerly NLPHL) classification
• Q-192 — Calcitriol-mediated hypercalcemia in lymphoma
• Q-197 — G-CSF + bleomycin combination

Sources