113.2 📚 國考版(醫師國考 / PGY OSCE)
113.2.0.1 📌 Cram Sheet
113.2.0.1.1 🔥 高 yield 15
- NHL 90% B-cell, 10% T/NK-cell;發生率為 HL 的 10 倍;> 40 歲指數 ↑
- DLBCL = 最常見 NHL(1/3);R-CHOP × 6 cure 65–70%;IPI ≥ 2 改 Pola-R-CHP(POLARIX)
- FL = 第二常見 indolent;t(14;18) BCL2/IgH;advanced asymptomatic → W&W;BR or R² 是 frontline
- Burkitt’s = 最快(doubling < 24h);t(8;14) MYC/IgH;Starry sky;DA-EPOCH-R / CODOX-M-IVAC + IT MTX + TLS prophylaxis;80–90% cure
- MCL t(11;14) cyclin D1+ CD5+ CD23−;TRIANGLE → ASCT 可省;BTK i + Tecartus CAR-T
- MALT lymphoma 與 H. pylori 強關聯;t(11;18) API2/MALT1 對 H. pylori 治療不敏感
- ATLL HTLV-1 driven(日本西南、加勒比);母乳傳染;flower cell + 高血鈣
- NK/T-cell nasal EBV-driven;亞洲 / 拉丁美洲;SMILE + RT;plasma EBV DNA monitor
- ALCL CD30+;ALK+(年輕、好預後 OS 82%)vs ALK−(差);Brentuximab + CHP (ECHELON-2) for CD30+ T-NHL
- Mycosis fungoides = CTCL CD4+;皮膚 patch → plaque → tumor;Sézary 是 erythrodermic 變體
- AITL polyclonal hypergamma + Coombs+ + 皮疹;CD3+ CXCL13+ PD-1+;TFH origin;TET2/DNMT3A/IDH2
- Waldenström’s IgM + hyperviscosity → plasmapheresis 急救;MYD88 L265P > 90%
- HL 與 NHL 鑑別:RS cell, B sx 多, contiguous spread, 80–85% cure with ABVD or BV-AVD
- Excisional biopsy is gold standard(不要 FNA)
- Pre-rituximab 必查 HBV(reactivation 可致命)
113.2.0.1.2 🔢 必背數字
| 項目 | 數字 |
|---|---|
| 美國年發病例 | 80,550 |
| NHL : HL 比 | 10:1 |
| B vs T 比 | 90 : 10 |
| DLBCL frontline cure | 65–70% |
| FL median OS | 15–20 yr |
| FL 轉 DLBCL 速率 | ~ 3% / yr |
| Burkitt doubling time | < 24 hr |
| Burkitt cure rate (treated) | 80–90% |
| ALK+ ALCL 8-yr OS | 82% |
| ALK− ALCL 8-yr OS | 49% |
| MCL median OS (classic) | 5–10 yr |
| MCL blastoid OS | 18 mo |
| ATLL median OS(acute) | 6 mo |
| Splenic MZL 5-yr OS | 77% |
| Waldenström’s IgM 在 intravascular % | 85%(plasmapheresis 有效原因) |
113.2.0.2 ⭐ 高 yield 表
113.2.0.2.1 Translocations / 分子標記
| 變異 | 基因 | 病種 |
|---|---|---|
| t(8;14)(q24;q32) | MYC/IgH | Burkitt’s |
| t(14;18)(q32;q21) | BCL2/IgH | Follicular |
| t(11;14)(q13;q32) | Cyclin D1/IgH | Mantle Cell |
| t(11;18)(q21;q21) | API2/MALT1 | MALT(H. pylori-resistant) |
| t(2;5)(p23;q35) | NPM1/ALK | ALK+ ALCL |
| t(3;14) | BCL6/IgH | DLBCL / FL |
| MYC + BCL2 (± BCL6) | rearrangements | Double/Triple-hit HGBL (poor) |
| MYD88 L265P | mutation | Waldenström’s / LPL, ABC DLBCL |
113.2.0.2.2 IHC 鑑別
| 病 | CD5 | CD10 | CD20 | CD23 | Cyclin D1 | Other |
|---|---|---|---|---|---|---|
| CLL/SLL | + | − | dim+ | + | − | CD200+, sIg dim |
| MCL | + | − | mod/bright | − | + | SOX11+ |
| FL | − | + | + | + | − | BCL2+, BCL6+ |
| DLBCL | − | ±(GCB) | + | − | − | BCL6 ±, MUM1 ±(ABC) |
| Burkitt’s | − | + | + | − | − | BCL2−, c-MYC+, Ki67 ~100% |
| MZL/MALT | −/+ | − | + | − | − | Lymphoepithelial lesions |
| HCL | − | − | + | − | − | CD25+ CD103+ annexin A1+ |
| AITL | (T+) | + | − | − | − | CD3+ CD4+ CXCL13+ PD-1+ BCL6+ |
| ALCL | (T+) | − | − | − | − | CD30+ ± ALK |
113.2.0.2.3 IPI (DLBCL)
| 因子 | 不利 | 分 |
|---|---|---|
| Age | ≥ 60 | 1 |
| LDH | > ULN | 1 |
| ECOG | ≥ 2 | 1 |
| Stage | III/IV | 1 |
| Extranodal sites | > 1 | 1 |
| Score | 風險 | 5-yr OS pre-R | 4-yr PFS post-R-CHOP |
|---|---|---|---|
| 0–1 | Low | 73% | 94% |
| 2 | Low-int | 51% | 80% |
| 3 | High-int | 43% | 53% |
| 4–5 | High | 26% | 53% |
113.2.0.2.4 FLIPI (FL)
| 因子 | 不利 |
|---|---|
| Age | > 60 |
| Stage | III/IV |
| Nodal sites | > 4 |
| LDH | > ULN |
| Hb | < 12 |
| Score | 10-yr OS |
|---|---|
| 0–1 | 71% |
| 2 | 51% |
| ≥ 3 | 36% |
113.2.0.2.5 Ann Arbor Staging
| Stage | 描述 |
|---|---|
| I | Single LN region or 1 extranodal site (IE) |
| II | ≥ 2 LN regions on same side of diaphragm (IIE if + adjacent extranodal) |
| III | LN regions on both sides of diaphragm (IIIS if + spleen) |
| IV | Diffuse / disseminated extranodal involvement |
| A / B | A = no B sx;B = B sx |
113.2.0.2.6 治療 backbone(記憶要點)
| 病 | Frontline |
|---|---|
| DLBCL | R-CHOP × 6 or Pola-R-CHP (POLARIX, IPI ≥ 2) |
| Early DLBCL favorable | R-CHOP × 4 (FLYER) ± IFRT |
| PMBCL / double-hit | DA-EPOCH-R |
| FL low-vol | Rituximab 單藥 |
| FL high-vol | BR or R-CHOP or R² |
| Burkitt’s | DA-EPOCH-R or CODOX-M/IVAC + IT MTX + TLS prophy |
| MCL young fit | R-Hyper-CVAD + cytarabine + ibrutinib (TRIANGLE) |
| MCL elderly | BR ± ibrutinib (SHINE) |
| MALT (H. pylori+) | H. pylori 治療(注意 t(11;18) 不應) |
| MALT 局部 | IFRT |
| Splenic MZL | Splenectomy or rituximab; HCV 治療 if HCV+ |
| Waldenström’s | Rituximab ± BR; ibrutinib + R; bortezomib |
| Mycosis fungoides | Topical / PUVA / EBT / mogamulizumab (advanced) |
| PTCL NOS | CHOP / CHOEP / brentuximab + CHP (CD30+) |
| ALK+ ALCL | CHOP / CHOEP(已較好預後) |
| ALK− ALCL CD30+ | Brentuximab + CHP (ECHELON-2) |
| ATLL acute | Chemo + zidovudine + IFN-α / mogamulizumab → allo-SCT |
| NK/T nasal | SMILE / DeVIC + RT(含 high-dose MTX + Asparaginase) |
113.2.0.2.7 22E 新藥 / 新標準
| 藥物 / 試驗 | Indication | 核心訊息 |
|---|---|---|
| POLARIX (Pola-R-CHP) | DLBCL frontline IPI ≥ 2 | PFS 優於 R-CHOP |
| ZUMA-7 (axi-cel) | DLBCL primary refractory / relapse < 12 mo | CAR-T 勝 ASCT |
| TRANSFORM (liso-cel) | 同上 | 同上 |
| Mosunetuzumab | FL 3rd-line | CD20×CD3 BiTE; 較 CAR-T 低 toxicity |
| Epcoritamab / Glofitamab | DLBCL R/R | CD20×CD3 BiTE; 包括 post-CAR-T |
| Tafasitamab + Lenalidomide | DLBCL R/R 不適 ASCT | CD19 mAb |
| Loncastuximab | DLBCL R/R | CD19-PBD ADC |
| TRIANGLE | MCL | Ibrutinib 加入 induction → ASCT 可省 |
| ECHELON-2 | CD30+ T-NHL | Brentuximab + CHP > CHOP |
| Brexucabtagene (Tecartus, ZUMA-2) | MCL R/R | CD19 CAR-T |
| Pirtobrutinib (BRUIN-MCL) | MCL post-cBTK | Noncovalent BTK |
113.2.0.3 🎯 自我檢測 20 題
- NHL 最常見 subtype? → DLBCL(~ 1/3)
- NHL 第二常見 indolent? → FL
- FL 標誌 translocation? → t(14;18) BCL2/IgH
- Burkitt’s 標誌 translocation? → t(8;14) MYC/IgH
- MCL 標誌 translocation? → t(11;14) cyclin D1/IgH
- ALK+ ALCL translocation? → t(2;5) NPM1-ALK
- MALT lymphoma 對 H. pylori 治療不應的 translocation? → t(11;18) API2/MALT1
- DLBCL frontline standard? → R-CHOP × 6;IPI ≥ 2 → Pola-R-CHP (POLARIX)
- Primary mediastinal large B-cell lymphoma 用什麼? → DA-EPOCH-R
- Double-hit DLBCL 預後? → median OS 12–18 mo(給 DA-EPOCH-R)
- Burkitt’s frontline + 必做? → DA-EPOCH-R / CODOX-M-IVAC + IT MTX + TLS prophylaxis
- MCL TRIANGLE 試驗結論? → 加 ibrutinib 進 induction → ASCT 不再 add benefit
- MCL relapsed CAR-T? → Brexucabtagene autoleucel (Tecartus)
- FL CAR-T 與 BiTE approved? → axi-cel, tisa-cel, mosunetuzumab (CD20×CD3)
- Splenic MZL 與哪個 virus 關聯? → HCV(HCV 治癒可使 MZL 退化)
- NK/T nasal 與哪個 virus? → EBV(plasma EBV DNA 是 marker)
- ATLL 與哪個 virus? → HTLV-1(母乳傳染)
- AITL 是什麼起源 T-cell? → Follicular helper T-cell (TFH);CD3+ CD4+ CXCL13+ PD-1+
- CD30+ T-NHL frontline 新標準? → Brentuximab + CHP (ECHELON-2)
- Pre-rituximab 必查? → HBV(reactivation 可致命)
113.2.0.4 🩺 PGY OSCE 場景
113.2.0.4.1 Scenario 1:60 歲男性 cervical LAD 4 cm + 進行性 + B sx
- Workup:
- 詳細病史(B sx + 暴露 + 免疫狀態)
- Excisional biopsy(禁 FNA)
- CBC, LDH, β2M, SPEP, HIV/HBV/HCV 篩檢
- PET-CT staging
- Marrow biopsy(特定 indication)
- LP(high CNS risk site / Burkitt-like / 廣 marrow involvement)
- DLBCL 確診 → IPI 評估 → R-CHOP / Pola-R-CHP
113.2.0.4.2 Scenario 2:70 歲女多 painless LAD + Hb 10 + LDH normal + 無 B sx
- → 切片 → FL grade 1–2
- FLIPI 計分;多 nodal sites + Hb < 12 + age > 60 → high-risk FLIPI
- 若無 organ dysfunction + asymptomatic → W&W
- 衛教 transformation warning signs(rapid LAD ↑, B sx, LDH ↑)
113.2.0.4.3 Scenario 3:35 歲男 abdominal mass + 嚴重 B sx + LDH 5x ULN + uric acid 高
- → Burkitt’s lymphoma 高度懷疑
- 立即 TLS prophylaxis(aggressive hydration + rasburicase + allopurinol)
- 監測 K, P, Ca, urate, Cr q6–8h
- 轉送 tertiary center for DA-EPOCH-R / CODOX-M-IVAC + IT MTX
- 不要拖延 — 一天就 doubles