第152 次è¯åˆå¸術研討會時間:民國101 å¹´12 月15 ... - å°ç£兒科醫å¸會
第152 次è¯åˆå¸術研討會時間:民國101 å¹´12 月15 ... - å°ç£兒科醫å¸會
第152 次è¯åˆå¸術研討會時間:民國101 å¹´12 月15 ... - å°ç£兒科醫å¸會
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第 152 次 聯 合 學 術 研 討 會<br />
時 間 : 民 國 101 年 12 月 15 日 ( 星 期 六 )<br />
地 點 : 中 國 醫 藥 大 學 附 設 醫 院 癌 症 大 樓 1 樓 階 梯 教 室<br />
會 長 : 葉 士 芃 主 任<br />
時 間<br />
演 講 題 目<br />
13:00 起 報 到<br />
13:30-13:35 會 長 葉 士 芃 主 任 致 歡 迎 詞<br />
13:35-13:40 中 華 民 國 血 液 病 學 會 田 蕙 芬 理 事 長 致 開 幕 詞<br />
專 題 演 講 主 持 人 :<br />
13:40-14:30 講 題 :Latest Topics of HSCT for AML – Fine Tuning of Reduced Intensity<br />
and Reduced Toxicity Conditioning for Allogeneic Stem Cell<br />
Transplantation in AML<br />
主 講 人 :Professor Arnon Nagler, MD, MSc<br />
會 員 演 講 (I) 主 持 人 :<br />
14:30-14:40 〔01〕A Chimeric Receptor with NKG2D Specificity Enhances Natural Killer<br />
Cell Activation and Killing of Tumor Cells<br />
張 裕 享 / 高 雄 榮 民 總 醫 院 小 兒 血 液 腫 瘤 科<br />
14:40-14:50 〔02〕Haploidentical Transplant Is Feasible when Using Combined BM and<br />
PBSC as Stem Cells Source: A Case Experience at NTUH<br />
黃 威 翰 / 台 大 醫 院 內 科 部 血 液 科<br />
14:50-15:00 〔03〕Monoclonal Gammopathy of Undetermined Significance Related Pyoderma<br />
Gangrenosum Refractory to Autologous Peripheral Blood Stem Cell<br />
Transplantation Was Treated with Allogenous Peripheral Blood Cell<br />
Transplantation<br />
陳 彥 谷 / 台 中 榮 民 總 醫 院 血 液 腫 瘤 科<br />
15:00-15:20 Coffee Break<br />
會 員 演 講 (II) 主 持 人 :<br />
15:20-15:30 〔04〕Diffuse Large B-cell Lymphoma of Testis in CGMH<br />
施 宣 任 / 林 口 長 庚 紀 念 醫 院 血 液 腫 瘤 科<br />
15:30-15:40 〔05〕FcgRIIIa 158 V/F Polymorphism Correlated with Rituximab-induced Late<br />
Onset Neutropenia in Newly-diagnosed B-cell Lymphoma but Not<br />
Correlated with Long-term Survival: A prospective study of Single<br />
Institute, Ten-year Follow-up<br />
賴 泓 誌 / 大 林 慈 濟 醫 院 血 液 腫 瘤 科<br />
15:40-15:50 〔06〕Synchronous Primary Brain Large B-cell Lymphoma and Metastatic Renal<br />
Cell Carcinoma<br />
盧 介 聖 / 三 軍 總 醫 院 血 液 腫 瘤 科
15:50-16:00 〔07〕Small Lymphocytic Lymphoma Complicated with Chylothorax: Case<br />
Report and Review of the Literature<br />
許 彥 寧 / 中 山 醫 學 大 學 附 設 醫 院 血 液 科<br />
16:00-16:10 〔08〕Chronic Lymphocytic Leukemia Cells in Ascites Detected by Clonality<br />
Analysis Using Gene Rearrangement Assay<br />
黃 彥 閔 / 林 口 長 庚 紀 念 醫 院 血 液 腫 瘤 科<br />
16:10-16:20 〔09〕Influence of Gender on the Prognosis on Adult Patients with Acute Myeloid<br />
Leukemia<br />
連 銘 渝 / 中 國 醫 藥 大 學 附 設 醫 院 血 液 腫 瘤 科<br />
備 註 :<br />
會 員 演 講 (III) 主 持 人 :<br />
16:20-16:30 〔10〕Primary Myeloid Sarcoma of Right Neck<br />
曾 瑞 如 / 台 中 榮 民 總 醫 院 小 兒 血 液 腫 瘤 科<br />
16:30-16:40 〔11〕Multiple Myeloma with Unusual Clinical Presentation<br />
蔡 明 宏 / 中 山 醫 學 大 學 附 設 醫 院 血 液 科<br />
16:40-16:50 〔12〕Pulmonary Embolism after Vertebroplasty in Patient with Multiple<br />
Myeloma<br />
周 政 緯 / 台 中 榮 民 總 醫 院 血 液 腫 瘤 科<br />
16:50-17:00 〔13〕Adherence to Deferasirox Treatment for Low Risk Myelodysplastic<br />
Syndrome with Hematological Improvement<br />
賴 冠 銘 / 彰 化 基 督 教 醫 院 血 液 腫 瘤 科<br />
17:00-17:10 〔14〕Hepatitis B Virus Reactivation in a Chronic Myeloid Leukemia Patient<br />
Receiving Imatinib<br />
李 品 萱 / 彰 化 基 督 教 醫 院 血 液 腫 瘤 科<br />
17:10-17:20 〔15〕Thrombotic Thrombocytopenic Purpura Presenting with Acute Myocardial<br />
Infarction: A Case Report<br />
賴 學 緯 / 三 軍 總 醫 院 血 液 腫 瘤 科<br />
17:20-17:25 中 華 民 國 血 液 及 骨 髓 移 植 學 會 林 凱 信 理 事 長 致 閉 幕 詞<br />
18:00- 晚 宴 ( 台 中 僑 園 大 飯 店 漂 亮 廳 )<br />
專 講 演 講 : 演 講 50 分 鐘 , 討 論 2 分 鐘 。47 分 鐘 鈴 響 一 聲 ,48 分 鐘 鈴 響 兩 聲 , 請 結 束 演 講 。<br />
會 員 演 講 : 每 題 演 講 8 分 鐘 , 討 論 2 分 鐘 。7 分 鐘 鈴 響 一 聲 ,8 分 鐘 鈴 響 兩 聲 , 請 結 束 演 講 。<br />
主 辦 單 位 : 中 華 民 國 血 液 病 學 會 、 中 華 民 國 血 液 及 骨 髓 移 植 學 會<br />
協 辦 單 位 : 中 國 醫 藥 大 學 附 設 醫 院<br />
贊 助 單 位 : 台 灣 協 和 醱 酵 麒 麟 股 份 有 限 公 司
CURRICULUM VITAE<br />
Professor Arnon Nagler, MD, MSc<br />
Arnon Nagler, MD, MSc, is Director of both the Division of Hematology and the<br />
Bone Marrow transplantation and Cord Blood Bank at the Chaim Sheba Medical<br />
Center, Tel Hashomer, Israel and Professor of Medicine at the Tel Aviv University, Tel<br />
Aviv, Israel.<br />
Dr Nagler received his medical training at the Hebrew University-Hadassah Medical<br />
School, Jerusalem, Israel, specializing in hematology at the Rambam Medical Center,<br />
Haifa, Israel. He carried out a Postdoctoral research fellowship in hematology and<br />
bone marrow transplantation at "Stanford University Hospital" Palo Alto, CA, in the<br />
USA, from 1986 to 1990.<br />
Dr. Nagler has been working in the fields of bone marrow transplantation for<br />
hematological malignancies, including NHL, and hemato-oncology, for the last 20<br />
years. His main clinical interests include stem cells, bone marrow transplantation,<br />
hematological malignancies, cord blood biology, and adoptive cell-mediated<br />
immunotherapy. Dr Nagler established the first public cord blood bank in Israel and<br />
performed the first cord blood transplantations from related and unrelated donors in<br />
genetic and malignant hematological disease in Israel. Dr Nagler is a member of<br />
multiple national and international societies and committees in the field. He serves on<br />
the Editorial Board of several journals and is a Section Editor for Leukemia. Dr<br />
Nagler has written numerous articles, reviews and chapters for peer-review journals,<br />
and is the principal investigator for a number of clinical studies. In addition, he has<br />
made numerous, invited, international presentations and has received several awards.
Latest Topics of HSCT for AML – Fine Tuning of Reduced Intensity and Reduced<br />
Toxicity Conditioning for Allogeneic Stem Cell Transplantation in AML<br />
Professor Arnon Nagler, MD, MSc<br />
待 補
〔01〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
一 個 具 有 NKG2D 專 一 性 的 嵌 合 受 體 加 強 自 然 殺 手 細 胞 之 活 化 和 腫 瘤 毒 殺<br />
A Chimeric Receptor with NKG2D Specificity Enhances Natural Killer Cell<br />
Activation and Killing of Tumor Cells<br />
原 著 (Original Article)<br />
Acute Leukemia<br />
張 裕 享<br />
1 , John Connolly 3 , Noriko Shimasaki 2 , Kousaku Mimura 4 , Koji Kono 4 , and<br />
Dario Campana 2<br />
Yu-Hsiang Chang 1 , John Connolly 3 , Noriko Shimasaki 2 , Kousaku Mimura 4 , Koji<br />
Kono 4 , and Dario Campana 2<br />
1<br />
高 雄 榮 總 兒 科 2 新 加 坡 大 學 兒 科 3 新 加 坡 免 疫 網 絡 ,A*STAR 4 新 加 坡 大 學 外 科<br />
Affiliations 1 Department of Pediatrics, Kaohsiung Veterans General Hospital1<br />
2<br />
Department of Pediatrics, National University of Singapore, Singapore 117599;<br />
3<br />
Singapore Immunology Network, A*STAR, Singapore 138648<br />
4<br />
Department of Surgery, National University of Singapore, Singapore 119228<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Purpose:<br />
We expressed this receptor(“NKG2D-DAP10-CD3zeta”) in human activated NK<br />
cells, and tested whether its supra-physiologic activating signals could augment tumor<br />
cell killing.<br />
Materials & Methods:<br />
After cloning the genes encoding these three molecules and assembling them into a<br />
single construct (NKG2D-DAP10-CD3zeta), we used a retroviral vector to express<br />
them in human NK cells.<br />
Results:<br />
Retroviral transduction of NKG2D-DAP10-CD3zeta markedly increased NKG2D<br />
surface expression in NK cells (n=21; P
〔02〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
以 合 併 骨 髓 及 周 邊 造 血 幹 細 胞 為 半 相 合 移 植 之 幹 細 胞 來 源 是 可 行 的 - 一 位 病 患 之 個<br />
案 報 告<br />
Haploidentical Transplant Is Feasible When Using Combined BM and PBSC as Stem<br />
Cells Source: A Case Experience at NTUH<br />
病 例 報 告 (Case Report)<br />
Hematopoietic Stem Cell Transplantation Including Stem Cell Biology<br />
黃 威 翰 、 李 啟 誠 、 姚 明 、 柯 博 升 、 林 建 廷 、 劉 家 豪 、 唐 季 祿<br />
Wei-Han Huang 1 , Chi-Cheng Li 1,2 , Ming Yao 1 , Bor-Sheng Ko 1 , Chien-Ting Lin 1,2 , Jia-<br />
Hau Liu 1,2 , Jih-Luh Tang 1,2<br />
1<br />
台 大 醫 院 內 科 部 血 液 科 2 台 灣 大 學 台 成 幹 細 胞 治 療 中 心<br />
Affiliations 1 Division of Hematology, Department of Internal Medicine, National Taiwan<br />
University Hospital 2 Tai-Cheng Stem Cell Therapy Center, National Taiwan University<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Haploidentical transplant<br />
Case presentation:<br />
A 55 years old woman underwent haploidentical transplantation with combining BMSC<br />
and PBSC<br />
Conclusions:<br />
Haploidentical transplant with combined BMSC and PBSC as stem cell source is<br />
feasible<br />
半 相 合 移 植 、 骨 髓 幹 細 胞 、 周 邊 造 血 幹 細 胞<br />
Haploidentical Transplant, Bone Marrow Stem Cells, Peripheral Blood Stem Cells<br />
著 作 權 授 權 同 意 書 : 本 人 不 同 意 將 本 次 投 稿 主 題 之 會 員 演 講 幻 燈 片 掛 網 。
〔03〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
在 未 知 臨 床 意 義 的 單 株 免 疫 球 蛋 白 增 高 合 併 壞 疽 性 膿 皮 病 的 病 患 接 受 異 體 幹 細 胞 移 植 可 改 善 皮 膚<br />
病 灶 及 血 液 中 免 疫 球 蛋 白 之 比 例<br />
Monoclonal Gammopathy of Undetermined Significance Related Pyoderma Gangrenosum Refractory to<br />
Autologous Peripheral Blood Stem Cell Transplantation Was Treated with Allogenous Peripheral Blood<br />
Cell Transplantation<br />
病 例 報 告 (Case Report)<br />
Hematopoietic Stem Cell Transplantation Including Stem Cell Biology<br />
陳 彥 谷 、 滕 傑 林 、 黃 文 豊<br />
Yeh-Ku Chen, Chieh-Lin Teng, Wen-Li Hwang<br />
台 中 榮 民 總 醫 院 血 液 腫 瘤 科<br />
Affiliations Division of Hematology/Oncology, Department of internal medicine of Taichung Veterans General<br />
Hospital<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Various kinds of skin disorders have been associated with monoclonal gammopathy. Some of these<br />
dermatologic disorders directly relate to the malignant plasma cells infiltration, but some are associated<br />
with the M protein. It is uncommon, however, that patients with MGUS manifest severe dermatologic<br />
diseases, especially pyoderma gangrenosum. Standard treatment to MGUS-related dermatologic<br />
disorders remains unclear.<br />
Case presentation:<br />
A 33-year-old man suffered from rapidly enlarging pustular papules and plagues that ulcerated the<br />
necrotic base over his left leg 5 years ago. His serum immuno-electrophoresis revealed IgA-λ<br />
monoclonal gammopathy (IgA 782 mg/dL, normal 70-400 mg/dL; κ/λ 1.17, normal 1.35-2.69). Bone<br />
marrow examination disclosed slight plasmacytosis, which was less than 10% of all the nucleated cells.<br />
Without anemia (Hb: 12.7 mg/dl; normal 12-14 mg/dl), thrombocytosis (platelet count 426 x 10^9 /l;<br />
normal 150-430 x 10^9 /l), osteolytic lesions, and impaired renal function, he was diagnosed as MGUS,<br />
IgA-λ, complicated with pyoderma gangrenosum.<br />
The patient was initially treated with methylprednisolone pulse therapy, but the response duration was<br />
short. He was then treated as having myeloma, but neither thalidomide nor chemotherapy with<br />
vincristine, adriamycin, and decadron regimen(VAD) was effective. High-dose cyclophosphamide<br />
(4g/M^2) was given, and his peripheral blood hematopoietic stem cells were collected. Consequently, he<br />
received autologous PBSCT with 6.5 x 10^ 6/kg CD34+ cells infusion. Conditioning regimen included<br />
total body irradiation(6 Gy) and melphalan(140 mg/M^2). After the PBSCT, his skin has been free of<br />
lesions for 18 months. Serum immuno-electrophoresis could not detect the IgA-λ M-protein either.<br />
18 months after autologous PBSCT , progressive swelling, erythematous change with pain were noted<br />
over right lower leg gradually. IgA level elevated, and then he received decadron with thalidomide for<br />
disease replase of MGUS. The next month, he received four cycles VTD regimen (Velcade and<br />
Thalidomide and Decadron), and good response for his skin lesion was noted. Then he received<br />
allogenous peripheral blood stem cell transplantation(PBSCT) (day on 2012/10/23) conditioned by RIC<br />
from a mis-matched unrelated donor, with engraftment (on 2012/10/31). Then he was followed up at<br />
hematologic clinic regularly.<br />
Conclusion:<br />
Since the pathogenesis of pyoderma gangrenosum basically comes from the plasma cells or secreted M-<br />
protein, it may be reasonable to treat this scenario as myeloma. Due to disease relapse even after<br />
autologous PBSCT., allogenous PBSCT might be one of the options for patient with monoclonal<br />
gammopathy-related dermatologic disorders refractory to steroid and chemotherapy.<br />
未 知 臨 床 意 義 的 單 株 免 疫 球 蛋 白 增 高 、 壞 疽 性 膿 皮 病 、 幹 細 胞 移 植<br />
Monoclonal Gammopathy of Undetermined Significance, Pyoderma Gangrenosum, Stem Cell<br />
Transplantation<br />
著 作 權 授 權 同 意 書 : 本 人 同 意 將 本 次 投 稿 主 題 之 會 員 演 講 幻 燈 片 轉 成 PDF 檔 格 式 後 , 掛 於 血 液 病 學 會 網 站 上<br />
供 該 會 會 員 瀏 覽 下 載 。
〔04〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
林 口 長 庚 醫 院 的 睪 丸 瀰 漫 性 大 B 細 胞 淋 巴 瘤<br />
Diffuse Large B-cell Lymphoma of Testis in CGMH<br />
原 著 (Original Article)<br />
Lymphoma, CLL, and Myeloma<br />
施 宣 任<br />
1 、 施 麗 雲<br />
1,2 、 王 博 南<br />
1 、 張 鴻<br />
1 、 吳 金 和<br />
1 、 郭 明 宗<br />
1 、 洪 玉 馨<br />
1 、 鄧 波<br />
Author Hsuan-Jen Shih 1 , Lee-Yung Shih 1,2 , Po-Nan Wang 1 , Hung Chang 1 , Jin-Hou Wu 1 ,<br />
Ming-Chung Kuo 1 , 1 Yu-Shin Hung, Po Dunn 1,2<br />
單 位<br />
1<br />
林 口 長 庚 紀 念 醫 院 血 液 腫 瘤 科 、 2 長 庚 大 學 醫 學 系<br />
Affiliations 1 Division of Hematology-Oncology, Department of Internal Medicine, Chang Gung<br />
Memorial Hospital, Taiwan<br />
2 Chang Gung University, College of Medicine, Taoyuan, Taiwan<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
We retrospectively reviewed 3,490 patients with non-Hodgkin's lymphoma diagnosed<br />
between Jan 1995 and Jun 2012 in Chang Gung Memorial Hospital in Linkou. The<br />
initial presentation was a testicular mass in twenty-two (0.6%) patients, all of whom<br />
had histologically proven lymphoma. Nineteen patients received orchiectomy while 3<br />
received biopsy alone. Two patients had Burkitt's lymphoma and one with diffuse<br />
large B-cell lymphoma (DLBCL) was lost to follow-up after diagnosis. Thus, 19<br />
patients with DLBCL were available for analysis. The median age was 69 years (range<br />
38.2 to 89.7 years). Sixteen patients had unilateral testis involvement (eight patients<br />
on each side). Three patients had bilateral testicular enlargement. The tumor stages<br />
according to the Ann Arbor system were IEA (10 cases), IIEA (5 cases), IIIEA (1<br />
case), IVA (2 cases), and IVB (1 case). The three patients in stage IV had extra-nodal<br />
involvement in skin, spine, and pleural effusion. The median overall survival (OS) and<br />
progression free survival (PFS) were 36.3 and 28.3 months for the 19 patients,<br />
respectively.<br />
Three stage IE patients received orchiectomy with or without scrotum radiotherapy<br />
(RT), and 16 patients received chemotherapy with or without orchiectomy or scrotum<br />
RT. Fifteen (78.9%) of the 19 patients achieved complete remission (CR) after initial<br />
treatment. The patients who achieved CR had a longer median OS and PFS than those<br />
who did not (60.1 vs. 3.0 months and 47.1 vs. 3.0 months, p < 0.001 and p < 0.001).<br />
Eight patients (53%) developed a recurrence. Two relapsed in inguinal lymph nodes, 1<br />
in retroperitoneal lymph node, brain, duodenum, kidney, soft tissue at back and bone<br />
marrow, and maxillary sinus each. Only 3 patients had achieved a second remission<br />
after salvage chemotherapy or local RT. None out of the 7 patients received<br />
prophylactic intrathecal chemotherapy (ITC) developed central nervous system (CNS)<br />
disease, in contrast, 1 out of the 12 patients who did not receive ITC developed CNS<br />
disease. Patients in low to low/intermediate International Prognostic Index (IPI) risk<br />
group had longer median OS and PFS (47.1 vs. 3.4 months, p = 0.04 and 28.3 vs. 3.4<br />
months, p = 0.04).<br />
In conclusion, similar to those reported in the literature, the testicular lymphoma is<br />
rare and affects elderly persons. The prognosis is poor because of the high relapse<br />
rate. Patients with testicular DLBCL have a better outcome if initial IPI score is low.<br />
瀰 漫 性 大 B 細 胞 淋 巴 瘤 、 睪 丸<br />
Diffuse Large B-cell Lymphoma, Testis<br />
著 作 權 授 權 同 意 書 : 本 人 不 同 意 將 本 次 投 稿 主 題 之 會 員 演 講 幻 燈 片 掛 網 。<br />
1,2
〔05〕 草 稿<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
FcgRIIIa 158 V/F 基 因 變 異 性 與 Rituximab 在 新 診 斷 之 B 細 胞 淋 巴 癌 引 起 之 晚 發<br />
性 白 血 球 低 下 相 關 , 但 與 長 期 存 活 情 形 無 相 關 性 - 單 一 醫 院 前 瞻 性 研 究 ,10 年 追 蹤<br />
報 告<br />
FcgRIIIa 158 V/F Polymorphism Correlated with Rituximab-induced Late Onset<br />
Neutropenia in Newly-diagnosed B-cell Lymphoma but Not Correlated with Longterm<br />
Survival: A prospective study of Single Institute, Ten-year Follow-up<br />
原 著 (Original Article)<br />
Lymphoma, CLL, and Myeloma<br />
賴 泓 誌<br />
1 、 李 思 錦<br />
1 、 余 芝 嘉<br />
3 、 蘇 裕 傑<br />
Author Hung-Chih Lai 1 , Szu-Chin Li 1 , Chi-Chia Yu 3 , Yu-Chieh Su 1,2*<br />
單 位<br />
1<br />
大 林 慈 濟 醫 院 血 液 腫 瘤 科 、 2 慈 濟 大 學 醫 學 院 、 3 嘉 義 大 學<br />
Affiliations 1 Division of Hematology–Oncology, Department of Internal Medicine, Buddhist<br />
2 School of Medicine, Tzu Chi University, Hualian, Taiwan<br />
3 Department of Biochemical Science and Technology, National Chiayi University,<br />
Abstract<br />
Chiayi, Taiwan<br />
1,2*<br />
後 補<br />
關 鍵 字<br />
Keyword<br />
基 因 多 型 性 , 莫 須 瘤 , 晚 發 性 白 血 球 低 下 症 , 長 期 存 活 情 形<br />
polymorphism, Rituximab, late onset neutropenia, long-term survival<br />
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〔06〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
原 發 性 腦 部 大 型 B 細 胞 淋 巴 瘤 與 轉 移 性 腎 細 胞 癌<br />
Synchronous Primary Brain Large B-cell Lymphoma and Metastatic Renal Cell<br />
Carcinoma<br />
病 例 報 告 (Case Report)<br />
Lymphoma, CLL, and Myeloma<br />
盧 介 聖 、 劉 智 軒 、 林 信 仲 、 戴 明 燊<br />
Cheih-Sheng Lu, Jhih-Syuan Liu, Hsin-Chung Lin, Ming-Shen Dai<br />
三 軍 總 醫 院 血 液 腫 瘤 科<br />
Affiliations Division of Hematology and Oncology, Department of Internal Medicine, Tri-Service<br />
General Hospital, National Defense Medical Center, Taipei, Taiwan<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Targeting PI3K/AKT/mTOR pathway in B-cell non-Hodgkin lymphomas (B-NHL) is<br />
important, and various small-molecule rapalogs focus on the prototype rapamycin<br />
including temsirolimus with less immunosuppression has been reported. This is the first<br />
report of single-agent activity of temsirolimus in patient with coexistence of renal cell<br />
carcinoma and central nervous system diffuse large B cell lymphoma.<br />
Case:<br />
A 73-year-old male patient presented with right-sided progressive hemiparesis. The<br />
magnetic resonance imaging (MRI) of brain showed a gadolinium-enhanced tumor in<br />
the left parietal lobe with significant perifocal edema. Besides, abdominal ultrasound<br />
indicated a heterogeneous echogenic lesion in right kidney. Emergent craniotomy with<br />
removal of brain tumor demonstrated a diffuse large B cell lymphoma. The follow-up<br />
magnetic resonance imaging with contrast of brain indicated a minimal residual tumor.<br />
Percutaneous biopsy of right renal tumor showed clear cell renal cell carcinoma.<br />
Coexistence of renal cell carcinoma and central nervous system diffuse large B cell<br />
lymphoma were diagnosed. His brain lymphoma did not respond to subsequent<br />
chemotherapy (Rituximab and high-dose methotrexate) and palliative irradiation. His<br />
renal tumor became larger during the period of chemotherapy. Targeted therapy with<br />
temsirolimus was initiated and the brain lesion showed apparent interval regression and<br />
stable disease in RCC. Unfortunately, the patient died of infectious complication 5<br />
months later.<br />
Conclusion:<br />
This is the first report of single-agent activity of temsirolimus in patient with<br />
coexistence of renal cell carcinoma and central nervous system diffuse large B cell<br />
lymphoma. Although the patient died secondary to sepsis, temsirolimus did control the<br />
synchronous double primary malignancies significantly. The obvious activity of<br />
monotherapy temsirolimus in patients with RC and central nervous system DLBCL is<br />
encouraging and promising strategy.<br />
特 癌 適 、 腎 細 胞 癌 、 瀰 漫 性 大 型 B 細 胞 淋 巴 瘤 、mTOR<br />
Temsirolimus, Renal Cell Carcinoma, Diffuse Large B Cell Lymphoma, mTOR<br />
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〔07〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
小 淋 巴 球 性 淋 巴 癌 合 併 乳 糜 胸 : 病 例 報 告 及 文 獻 回 顧<br />
Small Lymphocytic Lymphoma Complicated with Chylothorax: Case Report and<br />
Review of the Literature<br />
病 例 報 告 (Case Report)<br />
Lymphoma, CLL, and Myeloma<br />
許 彥 寧 、 黃 偉 修 、 蕭 培 靜<br />
Yen-Ning Hsu, Wei-Shou Hwang, Pei-Ching Hsiao<br />
中 山 醫 學 大 學 附 設 醫 院 內 科 部 血 液 腫 瘤 科 暨 中 山 醫 學 大 學 醫 學 系<br />
Affiliations Department of Hematology, Chung Shan Medical University Hospital and Chung<br />
Shan Medical University<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Chylothorax occurs when a disruption in the thoracic duct allows chyle to escape into<br />
the pleural space. The most commonly reported cause is malignancy, especially<br />
lymphoma. However, chylothorax caused by small lymphocytic lymphoma (SLL)is<br />
rarely reported in the literature.<br />
Case presentation:<br />
We describe a 50-year-old lady, suffered from oral ulcer and redness of eye 5 months<br />
ago, was found shortness of breath in admission. Chythorax was diagnosed by<br />
thoracocentesis, however, treatment was in vain despite NPO + TPN without lipid.<br />
History of low grade lymphoma was told later by the patient in 2004, and she was then<br />
found left perirenal and pararenal mass. Biopsy showed SLL. So chemotherapy with<br />
COP was tried. After 3 cycle of COP, follow up image showed decreased of the tumor<br />
size, but she suffered from persistent chythorax.<br />
Conclusion:<br />
The pathologenesis, diagnosis, treatment of chylothorax and the relationship between<br />
lymphoma and chylothorax are reviewed.<br />
小 淋 巴 球 性 淋 巴 癌 、 乳 糜 胸<br />
Small Lymphocytic Lymphoma, Chylothorax<br />
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〔08〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
以 基 因 重 組 方 法 診 斷 腹 水 中 慢 性 淋 巴 性 白 血 病 細 胞<br />
Chronic Lymphocytic Leukemia Cells in Ascites Detected by Clonality Analysis<br />
Using Gene Rearrangement Assay<br />
病 例 報 告 (Case Report)<br />
Lymphoma, CLL, and Myeloma<br />
黃 彥 閔 、 洪 玉 馨 、 施 麗 雲<br />
Yen-Min Huang, Yu-Shin Hung, Lee-Yung Shih<br />
林 口 長 庚 紀 念 醫 院 血 液 科<br />
Affiliations Division of Hematology, Department of Internal Medicine, Chang Gung Memorial<br />
Hospital, Linkou, Taiwan<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Ascites is an uncommon manifestation of chronic lymphocytic leukemia. The<br />
diagnosis of chronic lymphocytic leukemia (CLL) involvement is mainly based on<br />
morphologically and immunophenotypically similar to peripheral blood and bone<br />
marrow cells. However, it is relatively difficult to make a diagnosis of CLL<br />
involvement due to the pleomorphism of lymphocytes in ascites. Here we present a<br />
patient with diagnosis of CLL before. Three years after diagnosis, he suffered from<br />
massive transudative ascites. The etiology including heart, liver, or infection were<br />
surveyed but were all negative. Predominant T lymphocytes in ascites made it difficult<br />
to make immunophenotypical diagnosis by flow cytometry. We applied clonality<br />
analysis of immunoglobulin gene rearrangements on lymphocytes from ascites to<br />
diagnose the involvement of CLL, which was compatible with the pathological<br />
findings.<br />
慢 性 淋 巴 性 白 血 病 、 腹 水 、 基 因 重 組<br />
Chronic Lymphocytic Leukemia, Ascites, Gene Rearrangements<br />
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〔09〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
Influence of Gender on the Prognosis on Adult Patients with Acute Myeloid Leukemia<br />
Influence of Gender on the Prognosis on Adult Patients with Acute Myeloid Leukemia<br />
原 著 (Original Article)<br />
Acute Leukemia<br />
連 銘 渝 、 林 柏 翰 、 葉 士 芃 、 邱 昌 芳<br />
Ming-Yu Lien, Po-Han Lin, Su-Peng Yeh, Chang-Fang Chiu<br />
中 國 醫 藥 大 學 附 設 醫 院 血 液 腫 瘤 科<br />
Affiliations Division of Hematology and Oncology, Department of Internal Medicine<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Purposes:<br />
Prior studies suggested that hormone influence the leukemic cell survival, but the<br />
prognostic value of gender on the survival of adult acute myeloid leukemia (AML)<br />
was unknown. Therefore, from hypothesizing hormone may affect the prognosis of<br />
AML patients, we investigated the hypothesis that gender was associated the<br />
prognosis and the interaction between gender and the survival depends including, age,<br />
comorbidities, therapy methods, complications and period of diagnosis.<br />
Patients and Methods:<br />
We extracted data from the National Health Insurance system of Taiwan between<br />
2002 and 2010. A total of 6571 AML patients were enrolled into this study. Cox<br />
proportional hazards regression analysis was used to estimate the hazards ratios of<br />
overall survival (OS) and corresponding 95% confidence interval (CI) for controlling<br />
risks, including age, gender, comorbidities, therapy methods, complications and<br />
period of diagnosis.<br />
Results:<br />
Both in univariate and multivariate analysis, female patients had longer OS than male<br />
patients (univariate: hazard ratio [HR]=1.21, 95% CI, 1.14-1.28; multivariate:<br />
HR=1.10, 95% CI, 1.04-1.17). This result is compatible well with SEER Cancer<br />
statistics Review 1975-2009, which showed female patients had a significantly longer<br />
5-year relative survival than males in the period of 2002-2008 for Whites, Blacks and<br />
all races (survival rate of all: male vs. female, 21.9% vs. 27.3%, p
〔10〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
右 頸 部 原 發 性 髓 細 胞 肉 瘤 之 病 例 報 告<br />
Primary Myeloid Sarcoma of Right Neck<br />
病 例 報 告 (Case Report)<br />
Acute Leukemia<br />
曾 瑞 如 、 黃 芳 亮 、 張 德 高<br />
Jui-Ju Tseng, Fang-Liang Huang, Te-Kau Chang<br />
台 中 榮 民 總 醫 院 小 兒 血 液 腫 瘤 科<br />
Affiliations Department of Pediatric Oncology/Hematology, Taichung Veterans General Hospital,<br />
Taichung<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Isolated primary myeloid sarcoma without evidence of systemic malignant blasts is rare<br />
in pediatric oncology. Skin infiltration is the most frequent localization. Other sites are<br />
also reported which include reproductive organs, lymph nodes, soft tissues, orbits,<br />
CNS, bone, pericardium, and liver.<br />
Case presentation:<br />
A 13-years-old boy, a case of G6PD deficiency, was admitted to our hospital for<br />
progressive enlargement of right neck mass. He has history of right neck mass for one<br />
month, which was accompanied with hoarseness, dysphagia and difficulty in mouth<br />
opening. He denied weight loss, night sweating, or fever. During admission, a mass<br />
about 2 cm in size over right neck was revealed by neck CT. Initial blood studies were<br />
as followed: WBC: 5600/mm3 (neutrophil: 73.3 %, and lymphocyte: 19.5%),<br />
hemoglobin: 12.1 mg/dl, platelet: 268,000/mm3, sodium: 139 mEq/dl, potassium: 4.3<br />
mEq/dl, calcium: 9.4 mg/dl, uric acid: 4.4 mg/dl, LDH: 229 IU/L, GPT: 10 U/I and<br />
creatinine: 0.5 mg/dl. The result of biopsy of neck mass was diffuse infiltration of<br />
medium immature blast - like cell with immunohistochemical stain of focal positive in<br />
MPO and positive in LCA and negative in desmin and chromograin. The bone marrow<br />
biopsy was normal. Negative results of Image study with lung CT and whole body<br />
bone scan were noted. Isolated myeloid sarcoma over right neck was impressed.<br />
Chemotherapy with TPOG AML 97A was implanted. He was still having<br />
chemotherapy. Disease is under the status of complete remission.<br />
Conclusion:<br />
According to the experience of previous studies, patients with isolated primary myeloid<br />
sarcoma may subsequently developed acute myeloid leukemia, and the prognosis is<br />
poor. Intensive AML- specific chemotherapy is recommended after diagnosis. The role<br />
of HSCT and radiation therapy in the treatment of isolated primary myeloid sarcoma is<br />
still under investigated.<br />
原 發 性 髓 細 胞 肉 瘤 、 急 性 骨 髓 性 白 血 病<br />
Priamry Myeloid Sarcoma, Acute Myeloblastic Leukemia<br />
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〔11〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
罕 見 的 多 發 性 骨 髓 瘤 臨 床 表 現<br />
Multiple Myeloma with Unusual Clinical Presentation<br />
病 例 報 告 (Case Report)<br />
Lymphoma, CLL, and Myeloma<br />
蔡 明 宏 、 許 彥 寧 、 黃 偉 修 、 蕭 培 靜<br />
Ming-Hung Tsai, Yen-Ning Hsu, Wei-Shou Hwang, Pei-Ching Hsiao<br />
中 山 醫 學 大 學 附 設 醫 院 內 科 部 血 液 科<br />
Affiliations Division of Hematology, Department of Internal Medicine, Chung Shan Medical<br />
University Hospital<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
MM is characterized by the neoplastic proliferation of a single clone of plasma cells<br />
producing a monoclonal immunoglobulin. This clone of plasma cells proliferates in the<br />
bone marrow and often results in extensive skeletal destruction with osteolytic lesions,<br />
osteopenia, and/or pathologic fractures. The diagnosis of MM is often suspected<br />
because of bone pain with lytic lesions, increased total protein (monoclonal protein in<br />
the urine or serum), unexplained anemia, hypercalcemia, or acute renal failure.In our<br />
presentation, we describes three MM patients with unusual clinical presentation, which<br />
include left lung plasmacytoma combined with massive pleural effusion, rheumatoid<br />
arthritis-like polyarthritis and multiple intra-abdominal lymphoadenopathy with initial<br />
diagnosis of hepatocellular carcinoma. Pleural effusion in multiple myeloma occurs in<br />
about 6% of patients and is due to several etiologies requiring different types of<br />
therapy. These etiologies are most commonly, heart failure secondary to amyloidosis,<br />
followed by: pulmonary embolism, chronic renal failure, second neoplasm and pleural<br />
myelomatous involvement. Pleural effusion secondary to pleural involvement has been<br />
rarely reported in the literature but this condition as the first manifestation of multiple<br />
myeloma is exceptional. Rheumatoid arthritis -like polyarthritis is a very rare initial<br />
clinical manifestation of multiple myeloma, when serological marker of rheumatoid<br />
arthritis, such as FR and anti-CCP antibody are negative in patients with polyarthritis,<br />
cancer related arthritis might be a clue for differential diagnosis, such MM, MDS or<br />
lung cancer, and an intensive early cancer detection should be performed as soon as<br />
possible.<br />
多 發 性 骨 髓 瘤 、 關 節 炎 、 肋 膜 積 水 、 肝 癌<br />
Multiple Myeloma, Arthritis, Pleural Effusion, Heaptocellular Carcinoma<br />
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〔12〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
多 發 性 骨 髓 瘤 病 患 於 經 皮 穿 刺 椎 體 成 形 術 後 發 生 肺 栓 塞<br />
Pulmonary Embolism after Vertebroplasty in Patient with Multiple Myeloma<br />
病 例 報 告 (Case Report)<br />
Lymphoma, CLL, and Myeloma<br />
周 政 緯 、 黃 文 豊<br />
Cheng-Wei Chou, Wen-Li Hwang<br />
台 中 榮 民 總 醫 院 血 液 腫 瘤 科<br />
Affiliations Division of Hematology/Oncology. Department of internal medicine, Taichung<br />
Veterans General Hospital<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Percutaneous vertebroplasty for osteoporotic compression fracture or malignant<br />
osteolytic spinal tumor is a minimal invasive procedure to relieve pain and provide<br />
stability. Complication included leakage to spinal canal and perivertebral venous<br />
system. Pulmonary embolism after procedure was rare and most patient were<br />
asymptomatic.<br />
Case presentation:<br />
We report a 62-year-old woman with multiple myeloma IgG, kappa, Stage III(Durie-<br />
Salmon), received of vertebroplasty for multiple compression fracture of thoracolumbar<br />
spine(T12, L2, and L4). After procedure, shortness of breath as the first<br />
presenting symptoms was noted. Chest radiography shows numerous, radiographically<br />
dense tubular branching opacities over both lung fields. Chest computed tomography<br />
confirmed embolus of cement within the right upper and lower lobe segment artery,<br />
inferior vena cava and the paravertebral veins. Due to progressive dyspnea, she was<br />
referred to our hospital for evaluation. Respiratory failure with ventilator support was<br />
noted. Cardiologist and cardiovascular surgeon performed percutaneous intervention for<br />
pulmonary emboli retrieval with partially retrieval. However, this patient still died<br />
several days later.<br />
Conclusion:<br />
Percutaneous vertbroplasty for malignancy-related compression fracture could be<br />
relative a safe and minimal invasive procedure. But it could still cause fatal<br />
complication like pulmonary embolism. Further treatment plan for osteolytic spinal<br />
lesion should be further investigated.<br />
肺 栓 塞 、 多 發 性 骨 髓 瘤 , 經 皮 穿 刺 椎 體 成 形 術<br />
pulmonary embolism, multiple myeloma, percutaneous vertebroplasty<br />
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〔13〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
在 低 風 險 的 骨 髓 分 化 不 良 症 候 群 規 律 服 用 易 解 鐵 帶 來 血 液 相 的 改 善<br />
Adherence to Deferasirox Treatment for Low Risk Myelodysplastic Syndrome with<br />
Hematological Improvement<br />
病 例 報 告 (Case Report)<br />
Myelodysplastic Syndromes, Myeloproliferative Neoplasms<br />
賴 冠 銘 、 王 全 正 、 鐘 智 淵 、 林 炫 聿 、 林 敬 業 、 沈 銘 鏡 、 林 正 純 、 張 正 雄<br />
Guan Min Lai, Ciyuan-Jheng Wang , Chih-Yuan Chung , Shyuann-Yuh Lin, Chin-Ye<br />
Lin, Ming-Ching Shen, Jen-Tsun Lin, Cheng-Shyong Chang,<br />
彰 化 基 督 教 醫 院 內 科 部 血 液 腫 瘤 科<br />
Affiliations Division of Hemato-Oncology, Department of Internal Medicine, Chunghua Christian<br />
Hospital, Chunghua, Taiwan, R.O.C<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Most patients with myelodysplastic syndrome are transfusion-dependent. Iron overload<br />
resulted from transfusion may cause cardiac, hepatic, and endocrine dysfunction. In<br />
recent study, iron chelation therapy may decrease complication of iron overload in<br />
myelodysplastic syndrome. Interesting, iron chelation therapies also have effect on<br />
hematopoiesis and decrease frequency of blood transfusion and improve quality of life.<br />
Deferasirox (DFX) is an orally administered iron chelator which has been highly<br />
efficient in the treatment of secondary iron overload. Most frequent side effects of DFX<br />
are gastrointestinal disturbances, which leads in some patients to low adherence to the<br />
therapy. We will present a case and discuss about the effort and resources in strategies to<br />
improve adherence, those can result in significant long-term benefits.<br />
Case report:<br />
A 69 year-old-male was referred to our department due to thrombocytopenia. Physical<br />
examination revealed no hepatosplenomegaly. The initial blood counts were as follow:<br />
WBC: 2400/μL, Seg: 32%, Hb: 5.3 g/dl, and PLT: 6000/μL. Bone marrow aspiration<br />
revealed hypercellularity with RBC dysplasia, presence micromegakaryocyte, and
〔14〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
慢 性 骨 髓 性 白 血 病 病 人 在 使 用 基 利 克 之 後 引 起 B 型 肝 炎 活 化<br />
Hepatitis B Virus Reactivation in a Chronic Myeloid Leukemia Patient Receiving<br />
Imatinib<br />
病 例 報 告 (Case Report)<br />
Myelodysplastic Syndromes, Myeloproliferative Neoplasms<br />
李 品 萱 、 王 全 正 、 鐘 智 淵 、 林 炫 聿 、 林 敬 業 、 沈 銘 鏡 、 張 正 雄<br />
Ping-Hsuag Lee,Ciyuan-Jheng Wang,Chih-Yuan Chung,Shyuann-Yuh Lin, Chin-Ye<br />
Lin, Ming-Ching Shen,Cheng-Shyong Chang,<br />
彰 化 基 督 教 醫 院 內 科 部 血 液 腫 瘤 科<br />
Affiliations Division of Hemato-Oncology, Department of Internal Medicine, Chunghua Christian<br />
Hospital, Chunghua, Taiwan, R.O.C<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Hepatitis B virus (HBV) reactivation is a well-recognized complication in patients<br />
with chronic HBV infection receiving cytotoxic or immunosuppressive chemotherapy<br />
for hematologic malignancies. Imatinib mesylate (IM) (Glivec; Novartis, Basel,<br />
Switzerland), a selective Bcr/Abl tyrosine kinase inhibitor (TKI), is now widely used<br />
in the treatment of chronic myeloid leukemia (CML) and gastrointestinal stromal<br />
tumors. We report here one case of HBV reactivation during treatment of CML<br />
respectively with imatinib mesylate.<br />
Case report:<br />
A 43-year-old man was a HBV carrier who received regular follow-ups at our<br />
hospital. He was diagnosed with chronic myeloid leukemia during follow-ups in<br />
Division of Hepatology. He started to receive imatinib mesylate 400 mg once daily<br />
after bone marrow study. Complete cytogenic response was achieved after imatinib<br />
mesylate treatment. His liver tests after three months of treatment were within normal<br />
limits. However, he started to feel easy fatigue. The patient denied usage of other<br />
medications including acetaminophen or herbs. Laboratory investigation revealed an<br />
increased AST level of 273 U/L and an increased ALT level of 1086 U/L. HBV DNA<br />
was positive at a concentration of 229,254,031 IU/ml. Results for Hepatitis A IgM<br />
antibody, hepatitis C antibody, HSV, EBV, and CMV were negative. ANA and<br />
immunoglobulins were within normal limits. Liver ultrasonography showed mild<br />
coarse liver parenchyma and normal biliary tract. Because HBV reactivation was<br />
considered, he started to receive entecavir 0.5mg once daily. After 2-weeks treatment<br />
with entecavir, his ALT and AST levels fell to 891 and 82 IU/l respectively. Clinical<br />
symptoms and laboratory abnormalities resolved 6 weeks later. He got major<br />
molecular response status 8 months later with imatinib mesylate .<br />
Conclusion:<br />
This case report highlights the importance that HBV reactivation may occur in<br />
hematologic patients undergoing TKI therapy. Once HBV reactivation is suspected<br />
during TKI treatment, early detection of HBV load and utilization of antiviral agent<br />
are suggested to achieve better clinical outcome.<br />
B 形 肝 炎 活 化 、 慢 性 骨 髓 性 白 血 病 、 基 利 克<br />
Hepatitis B Virus Reactivation, Chronic Myeloid Leukemia, Imatinib<br />
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〔15〕<br />
中 文 標 題<br />
Title<br />
性 質<br />
內 容<br />
作 者<br />
Author<br />
單 位<br />
血 栓 性 血 小 板 減 少 紫 斑 症 倂 急 性 心 肌 梗 塞 之 個 案 報 告<br />
Thrombotic Thrombocytopenic Purpura Presenting with Acute Myocardial Infarction: A<br />
Case Report<br />
病 例 報 告 (Case Report)<br />
Anemia and Other RBC Disorders<br />
賴 學 緯 、 葉 人 華 、 劉 益 昇 、 黃 子 權 、 陳 佳 宏 、 吳 宜 穎 、 張 平 穎 、 戴 明 燊 、 何 景 良 、 陳<br />
宇 欽 、 高 偉 堯<br />
Shiue-Wei Lai 1 ,Ren-Hua Ye 1 ,Yi-Shen Liu 1 ,Tzu-Chuan Huang 1 ,Jia-Hong Chen 1 , Yi-Ying<br />
Wu 1 ,Ping-Ying Chang 1 ,Ming-Shen Dai1,Ching-Liang Ho 1 , Yeu-Chin Chen1,Wei-Yau<br />
Kao1 2<br />
1<br />
三 軍 總 醫 院 血 液 腫 瘤 科 、 2 新 店 慈 濟 醫 院 血 液 腫 瘤 科<br />
Affiliations 1 Division of Hematology-Oncology, Department of Medicine, Tri-Service General Hospital,<br />
National Defense Medical Center 2 Division of Hematology-Oncology, Department of<br />
Medicine, Xindian Tzu Chi General Hospital<br />
Abstract<br />
關 鍵 字<br />
Keyword<br />
Introduction:<br />
Thrombotic thrombocytopenic purpura (TTP) is acute disseminated microvascular<br />
thrombosis syndromes involving multiple organ systems and causing microangiopathic<br />
hemolytic anemia and thrombocytopenia. Cardiac involvement of TTP is less commonly<br />
encountered but carry high risk for mortality and morbidity.<br />
We report a case of TTP with presenting acute myocardial ischemia which leads to fatal<br />
consequence.<br />
Case presentation:<br />
A 69-year-old, male patient without systemic illness before, presented with progressively<br />
intermittent chest tightness for two days. The symptoms lasted for few minutes and relieved<br />
by rest. Her related manifestations included poor appetite, no fever or body weight loss. His<br />
electrocardiography (ECG) revealed ST-segment depression in leads V1 to V4. Laboratory<br />
work-ups on admission revealed white cell count 7.13 x 103/uL, hemoglobin 7.0 g/dL,<br />
platelet count 8 x 103 /uL, reticulocytosis (2.31%), schistocytes in the peripheral blood<br />
smear, prothrombin time 11.4 sec., activated partial thromboplastin time 29.3 sec.,<br />
fibrinogen 487.6 mg/dL, lupus anticoagulant less than 1.2, blood urea nitrogen 31.0 mg/dL,<br />
creatinine 1.3 mg/dL, total bilirubin 3.2 mg/dL, conjugated bilirubin 1.2 mg/dL, lactate<br />
dehydrogenase 1214 u/L, haptoglobin less than 5.8 g/L, creatinine kinase 544 u/L and<br />
troponin I 2.13 u/L. The echocardiography showed grade II diastolic function, 40% ejection<br />
fraction with generalized hypokinesia of of left ventricle and thrombus in apex (0.4x0.3cm).<br />
TTP complicating with acute non-ST elevation myocardial infarction was diagnosed. The<br />
cardiac biomarkers were elevated (CK 846 U/L, troponin I 8.44 U/L) despite of<br />
acetylsalicylic acid, clopidogrel and nitroglycerin treatment. The cardiac catheterization was<br />
not suggested due to bleeding risks. Urgent plasma exchange was performed and fatal<br />
cardiogenic shock developed due to deterioration of myocardial infarction.<br />
Conclusion:<br />
We present this report to highlight the difficult management for acute myocardial ischemia<br />
in TTP. In the review literature, early plasma exchange for TTP-related myocardial disease<br />
may reduce mortality and reverse ischemic cardiomyopathy.<br />
血 栓 性 血 小 板 減 少 紫 斑 症 、 微 小 血 管 內 溶 血 性 貧 血 、 冠 心 症 、 血 漿 置 換 術<br />
TTP, Microangiopathic Hemolytic Anemia, Coronary Artery Disease, Plasma Exchange<br />
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病 學 會 網 站 上 供 該 會 會 員 瀏 覽 下 載 。
中 華 民 國 血 液 病 學 會<br />
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