演題詳細

ポスター / Poster

ポスター 12 (Poster 12) :MDS:臨床 1

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F ロイトンホールABCD)
座長・司会
波多 智子 (Tomoko Hata):1
1:長崎大学原爆後障害医療研究所 血液内科学研究分野(原研内科)
 
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Myelodysplastic syndromes with del(13) in a patient with paroxysmal nocturnal hemoglobinuria (PNH)

演題番号 : PS-1-89

木村 哲 (Satoshi Kimura):1、野地 秀義 (Hideyoshi Noji):2、高橋 裕志 (Hiroshi Takahashi):2、木村 悟 (Satoru Kimura):1、斎藤 由理恵 (Yurie Saitoh):1、神林 裕行 (Hiroyuki Kanbayashi):1、松田 信 (Shin Matsuda):1、小田島 肇 (Hajime Odajima):3、竹石 恭知 (Yasuchika Takeishi):2、七島 勉 (Tsutomu Shichishima):2,4

1:Division of Hematology, Ohta-nishinouchi Hospital, Koriyama, Japan、2:Department of Cardiology and Hematology, Fukushima Medical University, Fukushima, Japan、3:Division of Pathology, Ohta-nishinouchi Hospital, Koriyama, Japan、4:Fukushima Research Institute of Environment and Medicine, Fukushima, Japan

 

The acquired bone marrow (BM) failure syndromes include aplastic anemia, PNH, and myelodysplastic syndromes (MDS). So far PNH patients with MDS at diagnosis have been sometimes reported. Here we describe a rare PNH patient who has complicated MDS with del(13q) during the clinical course. In 2004, a 53-year-old man had anemia and thrombocytopenia(Hb of 10.8 g/dL and PLT count of 49x109/L, respectively), 2.6% of CD55-CD59- erythrocytes and 3.3% of CD24- granulocytes (Gra) determined by flow cytometry, and hypocellular BM with normal karyotype of chromosome, indicating that he had PNH with hypocellular BM. In 2012, hematologic and laboratory examinations revealed that he had Hb of 10.3 g/dL, WBC of 2.4x109/L, PLT count of 66x109/L, reticulocyte count of 66x109/L, and LDH of 408 IU/L. The proportion of CD59- erythrocytes, FLAER- Gra, and FLAER- lymphocytes (Lym) by flow cytometry was 11.2%, 7.1%, and 3.3%, respectively. A BM examination showed hypercellularity with 0.8% and 56.0% of blasts and erythroblasts, respectively and chromosomal karyotype of 46,XY,del(13)(q14q32) in 20 cells, indicating that he also had MDS. Peripheral blood Gra and Lym were separated into the fractions of GPI+ and GPI- cells by FACSAria according to the expression with FLAER and analyzed by the FISH with probe of 13q14.3. The spots of del(13) were found in 93% of GPI+ Gra and in 1% and 4.5% of GPI+ and GPI- Lym, respectively, which are almost same proportions as those of cells from healthy individuals. Follow-up examinations with BM should be regularly done in PNH patients during the clinical courses.

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