PS-1-80 TAFRO syndrome in patient with rheumatoid arthritis

抄録 第 77 回日本血液学会学術集会 ポスター 第 77 回日本血液学会学術集会 16 ポスター ポスター 11 自己免疫疾患関連リンパ増殖異常症・TAFRO 2015 年 10 月 16 日(金) 16:50-17:50  ポスター会場①(石川県立音楽堂 B1F 交流ホール) ファシ...
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抄録 第 77 回日本血液学会学術集会

ポスター

第 77 回日本血液学会学術集会

16

ポスター

ポスター 11 自己免疫疾患関連リンパ増殖異常症・TAFRO 2015 年 10 月 16 日(金) 16:50-17:50  ポスター会場①(石川県立音楽堂 B1F 交流ホール) ファシリテーター:高井 和江 (Kazue Takai)(新潟市民病院 血液内科) ファシリテーター:鶴見 寿 (Hisashi Tsurumi)(岐阜大学大学院医学系研究科 血液病態学・臨床腫瘍学)

PS-1-80

TAFRO syndrome in patient with rheumatoid arthritis 中村 博 (Hiroshi Nakamura) 1 、北川 順一 (Junichi Kitagawa) 1 、後藤 尚絵 (Naoe Goto) 1 、 中村 信彦 (Nobuhiko Nakamura) 1 、二宮 空暢 (Soranobu Ninomiya) 1 、南谷 康仁 (Yasuhito Nannya) 1 、 原 武志 (Takeshi Hara) 1 、鶴見 寿 (Hisashi Tsurumi) 1 1:Dept.Hematol.,Gifu Univ.Grad.Sch.Med.,Gifu,Japan

Multicentric Castleman’s disease (MCD) is a polyclonal lymphoproliferative disorder that characterized by hypergammaglobulinemia, inflammation, thrombocytosis, and anemia. Recently, Takai et al. reported a new disease entity, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. In addition, Kojima et al. reported Japanese MCD cases with effusion and thrombocytopenia as Castleman-Kojima disease. Here, we report a patient with TAFRO syndrome. Case: A 57-year-old woman with rheumatoid arthritis, who had been received immunosuppressive agents including methotrexate since 10 years ago, referred to our hospital because of small lymphadenopathy, pleural effusion, pericardial effusion, and ascites. Discontinuation of immunosuppressive agents was not effective. Some malignant diseases and infections were excluded by various examinations including lymph node biopsy. After a month, the ascites developed with systemic lymphadenopathy, hepatosplenomegaly, anasarca, fever, anemia and thrombocytopenia. IL-6 level was increased in the serum and ascites (13pg/ml, 1900pg/ml, respectively). Bone marrow biopsy revealed mild myelofibrosis. He was suspected as having TAFRO syndrome. He received prednisolone (1mg/kg) and tocilizumab, an anti-interleukin-6 receptor antibody, and the inflammation findings such as fever and IL-6 level and thrombocytopenia were improved, but ascites remained. Further diagnostic and therapeutic strategy of this novel disease should be established through multicenter registry because of the rarity of TAFRO Syndrome.

  

第 77 回日本血液学会学術集会

17

ポスター 30

ポスター

MDS AZA (1) 2015 年 10 月 16 日(金) 16:50-17:50  ポスター会場②(ANA クラウンプラザホテル金沢 3F 鳳の間) ファシリテーター:金森 平和 (Heiwa Kanamori)(神奈川県立がんセンター 血液内科) ファシリテーター:久冨木 庸子 (Youko Kubuki)(宮崎大学医学部附属病院 輸血・細胞治療部)

PS-1-226

Chronic myelomonocytic leukemia treated with 5-azacitidine 馬渕 量子 (Ryoko Mabuchi) 1 、原 武志 (Takeshi Hara)

1,2

、鶴見 寿 (Hisashi Tsurumi)

1,2

、大山 正巳 (Masami Oyama)

1

1:Dept. of Int. Med., Kisogawa Municipal Hospital、2:Dept. Hematol., Gifu Univ. Grad. Sch. Med., Gifu, Japan

Background Chronic myelomonocytic leukemia (CMML) is a clinically heterogeneous disease, with no standard treatment.

We present the outcome of 3 patients diagnosed with CMML and treated with azacitidine in our institutions.

Case 1: A 72-year-old female was referred to our hospital with monocytosis and anemia. Cytogenetic analysis showed 47,XX,+1,der(1;7)(q10;p10),+8. A combined chemotherapy with cytarabine and etoposide was not effective. Azacitidine was subcutaneously administered 75 mg/m2 for 7 days every 28 days. She was able to become free of transfusion after 5 cycles of azacitidine treatment. Case 2: A 62-year-old male was referred to our hospital with monocytosis. Cytogenetic analysis showed 46,XY,i(17)(q10). A combined chemotherapy with cytarabine and etoposide was started, but CMML progressed. The patient treated with hydroxyurea to control monocytosis. He was able to reduce the dose of hydroxyurea after 3 cycles of azacitidine treatment. Case 3: A 76-year-old male was referred to our hospital with monocytosis and anemia. Cytogenetic analysis showed 46,XY,t(12;18)(p13;q21),idic(17)(p11.2). Although the patient had been initially treated with hydroxyurea, anemia progressed. Monocytosis and anemia was improved after 2 cycles of azacitidine treatment. Discussion No serious adverse events occurred in these 3 patients. Our experience of 3 CMML patients revealed the safety and utility of azacitidine for CMML patients. A large study is needed to identify in which conditions azacitidine may be useful in CMML.

  

第 77 回日本血液学会学術集会

18

ポスター

ポスター 47 AML (基礎) 2015 年 10 月 17 日(土) 17:10-18:10  ポスター会場①(石川県立音楽堂 B1F 交流ホール) ファシリテーター:小島 研介 (Kensuke Kojima)(佐賀大学医学部 血液・呼吸器・腫瘍内科) ファシリテーター:野阪 哲哉 (Tetsuya Nosaka)(三重大学大学院医学系研究科 感染症制御医学)

PS-2-6

Indoleamine 2,3-dioxygenase expression and serum L-kynurenine in acute myeloid leukemia 原 武志 (Takeshi Hara) 1 、二宮 空暢 (Soranobu Ninomiya) 1 、松本 拓郎 (Takurou Matsumoto) 1 、 柴田 悠平 (Yuhei Shibata) 1 、中村 信彦 (Nobuhiko Nakamura) 1 、中村 博 (Hiroshi Nakamura) 1 、 北川 順一 (Junichi Kitagawa) 1 、南谷 泰仁 (Yasuhito Nannya) 1 、鶴見 寿 (Hisashi Tsurumi) 1 1:Dept. Hematol., Gifu Univ. Grad. Sch. Med., Gifu, Japan

Background The powerful immunomodulatory effects of indoleamine 2,3-dioxygenase (IDO) are ascribed to its ability to catalyze the breakdown of the essential amino acid, L-tryptophan along the L-kynurenine pathway. The goal of this study was to investigate the role of L-kynurenine and IDO expression as a prognostic marker for acute myeloid leukemia (AML). Patients and methods We investigated 29 patients between December 2004 and September 2014 who were diagnosed with AML. L-kynurenine concentrations were measured by high-performance liquid chromatography. Bone marrow-derived mononuclear fractions were separated and IDO expression was analyzed by using extracted mRNA amplified by PCR. Results The median serum L-kynurenine level was 1.63 µM (range, 0.66-4.93 µM). The 3-year overall survival (OS) rates for all AML patients with L-kynurenine 2.4 µM were 76% and 0%, respectively (P < 0.0001). The 3-year OS rates for patients with and without IDO expression were 37% and 77%, respectively (P

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