T 細胞淋巴瘤

Buzzwords → Dx

Diagnostic Criteria

• WHO 5e / ICC 2022: large family — PTCL-NOS, ALK+ ALCL, ALK– ALCL, AITL/nodal TFH, ATLL, cutaneous (MF/SS, primary cut. ALCL, LyP), extranodal NK/T-cell, EATL/MEITL, hepatosplenic γδ, T-LGL, etc.
• Diagnosis by tissue biopsy with full T-cell IHC panel + TCR clonality.
• HTLV-1 serology in any aggressive PTCL with hypercalcemia or appropriate epidemiology.
• EBV in situ (EBER) for nasal-type, AITL, lymphomatoid granulomatosis.
• Staging: Lugano (Ann Arbor) for nodal; TNMB for MF/SS (skin patches/plaques/tumors, lymph nodes, viscera, blood).

Workup

• Excisional biopsy with full T-cell IHC + TCR γ/β clonality.
• PET-CT for staging.
• CBC, CMP, LDH, calcium, β2M, HTLV-1 (if hypercalcemia, lymphocytosis), HIV, HBV, HCV.
• CD30 IHC (drives brentuximab eligibility).
• EBER ISH for nasal-type and AITL.
• Marrow biopsy + flow for staging.
• Skin biopsy if cutaneous involvement (MF/SS).
• CSF analysis if neurologic symptoms.

Treatment Algorithm

flowchart TD
  A[Confirmed T-cell lymphoma] --> B{Subtype}
  B -- ALK+ ALCL --> C[CHOEP × 6<br>brentuximab + CHP if CD30+]
  B -- ALK– ALCL / AITL / PTCL-NOS<br>CD30+ --> D[Brentuximab + CHP × 6<br>ECHELON-2]
  D --> E[Auto-HCT consolidation in CR1 if fit<br>esp. AITL / PTCL-NOS]
  C --> F[Auto-HCT not standard if ALK+<br>only if relapsed]
  B -- ATLL --> G{Subtype}
  G -- acute / lymphoma --> H[mLSG15 / VCAP-AMP-VECP<br>+ allo-HCT in fit pts]
  G -- chronic / smoldering --> I[Watchful waiting<br>or interferon + zidovudine]
  B -- nasal NK/T --> J[SMILE or P-GemOx<br>+ involved-field RT]
  B -- MF / SS --> K{Stage}
  K -- early IA-IIA --> L[Skin-directed<br>topical steroids / NB-UVB / PUVA / topical mechlorethamine]
  K -- late stage / Sézary --> M[Systemic: bexarotene, mogamulizumab,<br>brentuximab if CD30+, romidepsin/vorinostat]
  M --> N[Allo-HCT for advanced<br>MF/SS in fit pts]
  D --> O{Relapse?}
  E --> O
  F --> O
  H --> O
  J --> O
  O -- yes, CD30+ --> P[Brentuximab vedotin]
  O -- yes, AITL --> Q[Romidepsin / azacitidine /<br>tipifarnib / belinostat]
  O -- yes, MF/SS --> R[Mogamulizumab anti-CCR4<br>or extracorporeal photopheresis]

Pearls / Pitfalls

• Brentuximab + CHP (ECHELON-2) is standard for CD30+ PTCL (all ALCL by definition; PTCL-NOS and AITL if CD30+) — beat CHOP for OS. Don't substitute vincristine for brentuximab in CD30+ disease.²
• ALK+ ALCL is the most curable PTCL (~80 %) — auto-HCT consolidation in CR1 is NOT standard for ALK+. ALK– ALCL, AITL, PTCL-NOS → auto-HCT in CR1 is appropriate.
• HTLV-1 + hypercalcemia + lytic bones + lymphocytosis with flower cells = ATLL acute type — treat with intensive chemo + allo-HCT; smoldering/chronic types observed.
• Mogamulizumab (anti-CCR4) approved for relapsed MF/SS — don't give immediately before allo-HCT (severe GVHD risk).
• Romidepsin approved for R/R PTCL and CTCL — HDAC inhibitor; QT prolongation, thrombocytopenia.
• Asparaginase-based regimens (SMILE) are key for NK/T-cell nasal-type — anthracyclines (CHOP) historically inferior. Asparaginase overcomes MDR1 efflux.
• Hepatosplenic γδ T-cell lymphoma is associated with chronic immunosuppression (e.g., infliximab + azathioprine for IBD) — aggressive, allo-HCT preferred.
• Lymphomatoid papulosis (LyP) is NOT treated as a lymphoma — it's a CD30+ self-limiting cutaneous condition; observe + topical therapy.
• MF/SS skin involvement TNMB staging — different from Lugano. Treat indolent stages with skin-directed (NB-UVB, PUVA, topical steroids/mechlorethamine, RT).
• Allo-HCT is the only curative option for advanced MF/SS, ATLL, hepatosplenic γδ.

Cross-references

• Cytogenetics Atlas — t(2;5) NPM-ALK, DUSP22, TP63
• Drug Regimens — CHOEP, BV-CHP, SMILE, mogamulizumab
• Allo-HCT for advanced T-cell
• Hypercalcemia — ATLL classic

相關題目

• Q-052 — T-cell — CD30+ peripheral T-cell lymphoma frontline
• Q-053 — T-cell — adult T-cell leukemia/lymphoma (ATLL) recognition
• Q-054 — T-cell — extranodal NK/T-cell nasal-type
• Q-141 — PRCA — T-LGL leukemia association

Sources