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Q1. Which statement about peripheral T-cell lymphoma (PTCL) is not true?

  • (A) Prognostic index for T-cell lymphoma” is an improved index developed specifically for PTCL.
  • (B) Brentuximab vedotin has demonstrated objective responses in more than 80 percent of patients with relapsed/refractory ALCL.
  • (C) Polyclonal hypergammaglobulinemia and autoimmune hemolytic anemia are rarely seen in angioimmunoblastic T-cell lymphoma.
  • (D) Adult T-cell leukemia/lymphoma is a mature CD4+CD25+ T-cells caused by infection with the retrovirus, HTLV-1.
點此顯示正解

(C) Polyclonal hypergammaglobulinemia and autoimmune hemolytic anemia are rarely seen in angioimmunoblastic T-cell lymphoma.

詳解

Option (C) is FALSE. Polyclonal hypergammaglobulinemia and autoimmune hemolytic anemia are common, not rare, in angioimmunoblastic T-cell lymphoma (AITL). Hypergammaglobulinemia occurs in approximately 30% of patients, and autoimmune hemolytic anemia (AIHA) is reported in 13-19% of patients24. Direct antiglobulin test (DAT) positivity is even more frequent, occurring in 32-58% of patients1. These autoimmune phenomena are characteristic features of AITL, arising from the tumor cells' origin as follicular helper T cells (TFH), which physiologically promote B-cell differentiation and antibody production134. The dysregulated B-cell activation in AITL leads to polyclonal hypergammaglobulinemia and production of autoantibodies, resulting in autoimmune cytopenias including warm/Coombs-positive AIHA15.

Why the other options are TRUE:

(A) Prognostic Index for T-cell lymphoma (PIT) is an improved index developed specifically for PTCL — TRUE. The PIT was developed specifically for peripheral T-cell lymphomas and includes four factors: age ≥60 years, performance status ≥2, elevated LDH, and bone marrow involvement2. This differs from the standard International Prognostic Index (IPI), which was originally developed for aggressive B-cell lymphomas and includes five factors (age, stage, LDH, extranodal sites, and performance status). The PIT has been validated as a prognostic tool specifically for PTCL patients2.

(B) Brentuximab vedotin has demonstrated objective responses in more than 80% of patients with relapsed/refractory ALCL — TRUE. The pivotal phase II study of brentuximab vedotin in relapsed/refractory systemic anaplastic large cell lymphoma (ALCL) demonstrated an overall objective response rate of 86% (50/58 patients), with complete remission achieved in 57%[^8]. Long-term follow-up data confirmed sustained responses, with median duration of response of 12.6 months and median duration of complete remission of 13.2 months[8][9]. These results were consistent across both ALK-positive and ALK-negative ALCL subtypes6.

(D) Adult T-cell leukemia/lymphoma is a mature CD4+CD25+ T-cell malignancy caused by infection with the retrovirus HTLV-1 — TRUE. Adult T-cell leukemia/lymphoma (ATLL) is caused by human T-lymphotropic virus type 1 (HTLV-1) infection and is characterized by malignant proliferation of mature CD4+ T cells that typically express CD25 (the IL-2 receptor alpha chain). HTLV-1 is a retrovirus endemic in certain geographic regions including Japan, the Caribbean, and parts of Africa and South America.

詳解 · 中文翻譯

選項 (C) 是假的。 多克隆過球蛋白血症和自身免疫溶血性貧血是常見的，而不是罕見的，在血管免疫母細胞性 T 細胞淋巴瘤（AITL）中。過球蛋白血症發生於約 30% 的患者，自身免疫溶血性貧血（AIHA）報告於 13-19% 的患者中24。直接抗球蛋白測試（DAT）陽性甚至更頻繁，發生於 32-58% 的患者中1。這些自身免疫現象是 AITL 的特徵特徵，起源於腫瘤細胞作為濾泡輔助 T 細胞（TFH），其在生理上促進 B 細胞分化和抗體產生134。AITL 中的失調 B 細胞激活導致多克隆過球蛋白血症和自身抗體產生，導致自身免疫細胞減少症包括溫/Coombs 陽性 AIHA15。

為什麼其他選項是真實的：

(A) T 細胞淋巴瘤預後指數（PIT）是專門為 PTCL 開發的改進指數 — 真實。PIT 是專門為周邊 T 細胞淋巴瘤開發並包括四個因素：年齡 ≥60 歲、性能狀態 ≥2、升高的 LDH 和骨髓受累2。這不同於標準國際預後指數（IPI），其最初為攻擊性 B 細胞淋巴瘤開發並包括五個因素（年齡、分期、LDH、結外位點和性能狀態）。PIT 已驗證作為預後工具專門用於 PTCL 患者2。

(B) Brentuximab vedotin 在復發/難治 ALCL 患者中已證示超過 80% 的客觀反應 — 真實。pivotal 第 II 期 brentuximab vedotin 在復發/難治系統性間變大細胞淋巴瘤（ALCL）中的研究證示 86% 的總客觀反應率（58 名患者中的 50 名），完全緩解在 57% 中達成[^8]。長期隨訪數據確認持續的反應，具有反應的中位持續時間 12.6 個月和完全緩解的中位持續時間 13.2 個月[8][9]。這些結果在 ALK 陽性和 ALK 陰性 ALCL 亞型中都持續6。

(D) 成人 T 細胞白血病/淋巴瘤是由 HTLV-1 視病毒感染引起的成熟 CD4+CD25+ T 細胞惡性腫瘤 — 真實。成人 T 細胞白血病/淋巴瘤（ATLL）由人 T 淋巴營養病毒 I 型（HTLV-1）感染引起並由特徵是通常表達 CD25（IL-2 受體 alpha 鏈）的成熟 CD4+ T 細胞的惡性增殖。HTLV-1 是在某些地理區域（包括日本、加勒比海和非洲和南美的部分）中流行的視病毒。

參考文獻 (AMA)

  1. Federico M, Rudiger T, Bellei M, et al. Clinicopathologic Characteristics of Angioimmunoblastic T-Cell Lymphoma: Analysis of the International Peripheral T-Cell Lymphoma Project. Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 2013;31(2):240-6. doi:10.1200/JCO.2011.37.3647. PMID:22869878. ↩↩↩↩↩↩

  2. Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH. Angioimmunoblastic T-Cell Lymphoma. Critical Reviews in Oncology/Hematology. 2008;68(3):264-71. doi:10.1016/j.critrevonc.2008.06.012. PMID:18684638. ↩↩↩↩↩↩

  3. Crickx E, Poullot E, Moulis G, et al. Clinical Spectrum, Evolution, and Management of Autoimmune Cytopenias Associated With Angioimmunoblastic T-Cell Lymphoma. European Journal of Haematology. 2019;103(1):35-42. doi:10.1111/ejh.13239. PMID:30985955. ↩↩

  4. Wei C, Li W, Qin L, et al. Clinicopathologic Characteristics, Outcomes, and Prognostic Factors of Angioimmunoblastic T-Cell Lymphoma in China. Cancer Medicine. 2023;12(4):3987-3998. doi:10.1002/cam4.5248. PMID:36106610. ↩↩↩↩

  5. Pro B, Advani R, Brice P, et al. Brentuximab Vedotin (SGN-35) in Patients With Relapsed or Refractory Systemic Anaplastic Large-Cell Lymphoma: Results of a Phase II Study. Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 2012;30(18):2190-6. doi:10.1200/JCO.2011.38.0402. PMID:22614995. ↩↩

  6. Pro B, Advani R, Brice P, et al. Five-Year Results of Brentuximab Vedotin in Patients With Relapsed or Refractory Systemic Anaplastic Large Cell Lymphoma. Blood. 2017;130(25):2709-2717. doi:10.1182/blood-2017-05-780049. PMID:28974506. ↩↩

  7. Ong SY, Zain JM. Aggressive T-Cell Lymphomas: 2024: Updates on Diagnosis, Risk Stratification, and Management. American Journal of Hematology. 2024;99(3):439-456. doi:10.1002/ajh.27165. PMID:38304959. ↩

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ORR: 86% J Clin Oncol. 2012 Jun 20;30(18):2190-6

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