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原文題目(出處): Lymphoma in Taiwan: Review of 1374 neoplasms from a single institution according to the 2016 Revision of the World Health Organization Classification

J Formosan Med Association 2017;116:620-5

原文作者姓名: Shih-Sung Chuang , Department of Pathology of Chi-Mei medical center, Tainan, Taiwan.

通訊作者學校: Department of pathology, National Taiwan University , Taipei, Taiwan

報告者姓名(組別): 吳上毛 A2

報告日期: 2017.08.08

內文:

A. Main Objectives :

a. Aimed at classifying lymphoid neoplasms in Taiwan

b. Compare the Frequency of different types among other nations.

B. Intro :

a. Studying 1347 cases from 2000~2016 from 奇美醫院

b. Characterization of Lymphoid neoplasm is hard and differs with technological advancement

c. 2008 WHO classification is standard now

d. Lymphoma types differs across nations and regions; reflects, race, social status, and environment factors.

e. In prior study :

i. T cell & NK T cell lymphoma is higher than western countries ii. Frequency of Follicular Lymphoma (FL) Increases in 2000’s in

Taiwan C. Materials & Methods

a. Diagnosed with 2008 WHO classification & 2016 revision b. Diagnosised with immunohistochemistry & flow cytometric

immunophenotyping

c. Divide into B cell type & T cell type with Clonality Assay d. B/T cell receptor rearrangement for 排除型態 mimics

e. In situ EBV mRNA 混合法用在 Peripheral T-cell lymphoma,排除 extranodal NK/T-cell lymphoma, nasal type

f. 使用 Fluorescene in situ 混合法,來鑑別 lymphoma 相關染色體 translocation ,其主要用於鑑別 Burkitt Lymphoma (BL)& High Grade B-cell Lymphoma, NOS(B-cell lymphoma, unclassifiable) g. Primary Splenic Lymphomas :

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Main: flow cytometric imunophenotyping & morphological appearance. Also:

i. Without surgical / biopsy 樣本 : Splenic B-cell lymphoma, unclassifiable

ii. Splenic Maginal Zone Lymophma(MZL), Hairy cell leukemia 等地切片中若沒有 Splenic 組織都會被分類至此

h. 在 ymphocytosis 的 case 中,卻沒有器官肥大(organomegaly)/未達 Chronic Lymphocytic Leukemia 的標準者,分類至 Unclassifiable Small B-cell Leukemia

i. Flow cytometric immunophenotyping and/or bone marrow 抽液診斷 為 Mature Small B-cell Leukemia,用以下方式再細分:

i. 區分 Plasmacytoid Lymphocytes:

1. 骨髓抹片中, plasmacytoid lymphocyte, plasma cell, small lymphocytes 型態

2. Plasmacytoid lymphocyte, plasma cell 的 Flow cytometric immunophenotyping ,與 Mature B-cell lymphoma 不同

3. 有 IgM , monoclonal Gamma-globulin

j. 轉變型 lymphoma 以最初的診斷為主;同時有兩種並存者,視為兩 種 lymphoma

D. Results

a. Hodgkin Lymphoma(HL) : 6.09%(82 case) b. Non-HL : 93.31 % (1257)

c. Other diseases : 0.59% (8)

i. Composite lymphoma : 3 cases

ii. Mediastinal lymphoblastic lymphoma(2) iii. Mediastinal gray zone lymphoma(1) iv. Folicullar dendritc cell sarcoma (1)

v. Langerhans cell neoplasm (1)

d. HL is 6 %(4~8% in Asia, greatly lower then west15~30%

e. 1 only of 82 nodular lymphocyte-predominant HL i. 99 % of HL are Classic HL(CHL)

f. Of 1257 cases:

i. B-cell : 82.66% (1039 cases)

1. No.1 : DLBCL(diffuse large B-cell lymphoma), FL(Follicular Lymphoma), MALT(Mucosa-associated lymphoid tissue) lymphoma

2. Account DLBCL+FL+MALT + MZL(marginal zone

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lymphoma) most common :

#1. DLBCL(50.62%)

#2. FL (16.27%),

#3. MZL (12.70%) 3. 2nd tier most common:

#1. CLL(small lymphocytic lymphoma)(7.31%)

#2. unclassifiable small B-cell(3.27%)

#3. Mantle cell lymphoma (2.5%)

#4. BL (2.41 %) ii. T-cell: 17.34 % (218 cases)

#1. Angioimmunoblastic T-cell lymphoma(AITL;18.3%)

#2. ENKTL(), nasal type(16.5%)

#3. PTCL-NOS(15.1%)

#4. Systemic anaplastic large cell lymphoma(ALCL,10.5%)

a. Anaplastic lymphoma kinase (ALK+; 5.0%) b. ALK- ; 5.5%

-2nd Tier most common T-cell lymphoma:

#1. T-cell large granular lymphocytic leukemia(T-GL leukemia; 7.3%)

#2. Adult T-cell Leukemia(4.1%)

#3. Mycosis Fungoides (4.1%)

#4. Primary cutaneous CD30+ T-cell lymphoproliferative disorder(4.1%)

a. Primary cutaneous ALCL(1.4%) b. Lymphomatoid papulosis(2.8%)

iii. 10 cases of 218 TL 被剃除,因沒有確切來源(nodal &

extranodal 並存) 剩餘 208 case 中:

1. Nodal (37%; 76cases)

a. AITL most common (53%; 40 cases) b. PTCL-NOS(21%, 16 cases)

c. Systemic ALCL[17%; 9% ALK(+) & 8%

ALK(-)]

2. Extranodal (63%, 132 cases) a. ENKTL(26.5%; 35 cases)

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b. T lymphoblastic Leukemia (13.6%; 18 cases) c. T-LGL leukemia (12.1%; 16 cases)

d. PTCL-NOS (7.6%; 10 cases) E. Discussion :

B-cell lymphoma:

a. 1347 cases of 16 years (2000~2016)

b. 跟過去研究資料一起看,跟西方比較起來:

i. HL 機率較低(%6)

ii. T-cell neoplasms 較高 (所有淋巴病變中 16%,所有 NHL 中 17%)

c. 1347 cases 中,有 8 cases (0.60%)同時有兩種並存

d. 西方主要的淋巴病變中,HL 佔了 15~30%, 其中的 90~95% Classic HL(CHL), 其餘為 Nodular lymphocyte predominant Hodgkin lymphoma(NLPHL; 3~8%)

e. CHL 在工業化國家較開發中國家多 f. HL 頻率在東亞國家較西方低很多 g. NLPHL 頻率比起西方是極低(1.2~3.7%) h. NLPHL 佔所有 HL 的比率也是在東方較低 i. DLBCL 最多,10 個 case 中會有 4~5 個 case

j. 東亞國家中,FL 在台灣(16%),日本(24%)是第二多

k. 根據一篇 1993~2012 台灣癌症登記資料庫的統計研究,FL 近期在 台灣出現頻率有變高,且有很強的 birth-cohort effect,原因未知 l. FL 在韓國(%3),中國(4%)很低,在兩國是 MALT lymphoma 第二

多 B-cell lymphoma type,可能與兩國有較高的 Helicobacter pylori 感染 gastric MALT lymphoma,胃癌胃鏡檢驗補助計畫有關

m. 在台灣,MZL 較低可能與腸胃科醫師在早期就幫病患消滅 H.plori 感染有關,而間接減少 gastric MALT lymphoma 的出現頻率

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T-cell lymphoma:

n. ENKTL(Extranodal NK/T-cell Lymphoma) 在中國意外的很常見,幾 乎佔所有 T-cell lymphoma 一半,在韓國也很常見(佔 1/3 的案例),

原因不明,但可能跟生活方式,環境因子有關(務農,接觸殺蟲劑;

住在焚化爐附近等)

o. EBV mRNA 混合法的成果,強烈顯示種族的相關性,代表了對 EBVmRNA 免疫反應有 defect 是由基因表現缺失造成

p. 在東亞國家,AITL 較 PTCL-NOS 少,但參考此次資料,AITL 近年在台灣增多

q. 在法國為例,AITL 是目前最多的,原因可能為對疾病有較多認 識,進而將其診斷為 AITL,而非誤診為不尋常免疫反應而已 F. 這篇研究的缺點:

a. 單一病理中心(奇美醫院)的 16 年的資料,可能無法反映全國的真 實分布狀態

b. 其中一位研究員在座 flow cytometric immunophenotypicng 時可能 取了較多的 Mature lymphoid leukemia 的 case

G. 這篇研究的優點:

a. 有長期追蹤的資料,其中一位 case 追蹤期長達 20 年,因而得以幫 助區分並存 lymphoid neoplasm 的 case

H. 結論:

a. 分析了全台灣最大的 lymphoma series b. 導論出 HL 是很稀有的(佔總體 6%)

c. T-cell neoplasms 佔所有 NHL 的 17 %,與其他東亞國家相近 d. 整體來說,在東方,西方比較下可觀察到,東方的 HL 頻率較高,

T-cell neoplasms 較低

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題號 題目

1 Which of the following lymphoma is the most common type ? (A) Hodgkin Lymphoma(HL)

(B) Non-Hodgkin Lymphoma(NHL) (C) Burkitt Lymphoma

(D) Follicular Lymphoma 答案

( B )

出處:Oral Pathology : Clinical Pathologic Correlations, 7th edition p. 228, 229

題號 題目

2 What are the NOT likely reasons for different percentage of certain lymphomas between geographical regions?

(A) Life style

(B) Environmental factors (C) Religion

(D) More understanding to the disease 答案

( C )

出處:Oral Pathology : Clinical Pathologic Correlations, 7th edition, p.238, 239

參考文獻

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