Analysis of the pathology and regulation system in pregnancy-associated thrombotic microangiopathy (TMA)

• Project/Area Number: 23591425
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Hematology
• Research Institution: Nara Medical University
• Principal Investigator: FUJIMURA Yoshihiro 奈良県立医科大学, 医学部, 名誉教授 (80118033)
• Co-Investigator(Kenkyū-buntansha): MATSUMOTO Masanori 奈良県立医科大学, 輸血部, 教授 (60316081) ; SADO Toshiyuki 奈良県立科大学, 産婦人科, 講師 (50275335) ; HAYAKAWA Masaki 奈良県立医科大学, 輸血部, 医員 (30516729)
• Project Period (FY): 2011-04-28 – 2015-03-31
• Project Status: Completed (Fiscal Year 2014)
• Budget Amount: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000); Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2011: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
• Keywords: 妊娠関連TMA / HELLP症候群 / ADAMTS13 / VEF / 胎盤由来E-NTPDase / VWF / 胎盤由来E-NTPase / 活性型VWF / 補体調節因子 / 妊娠中毒症 / 正常妊娠経過 / 胎盤E-NTPDase

Outline of Final Research Achievements

In this study, we aimed to reveal the pathology of pregnancy-associated thrombotic microangiopathy (TMA). We analyzed von Willebrand factor (VWF), VWF-cleaving specific cleavage protease ADAMTS13 and placenta-derived E-NTPDase by using the plasma samples of normal pregnant women and the patients with pregnancy-induced hypertension (PIH) or HELLP syndrome.
We identified that the patients with HELLP syndrome had the severe deletion of "VWF unbound forms of ADAMTS13 (free ADAMTS13)" in isoelectric focusing. We previously showed that cryosupernatant, which abundantly contains “free ADAMTS13”, inhibited initial phase of platelet aggregation. Thus, we suggested that the development of HELLP syndrome might be associated with the decreased function of inhibiting initial phase of platelet aggregation by “free ADAMTS13”.

Research Products

• [Journal Article] A unique case involving a female patient with Upshaw-Schulman syndrome: low titers of antibodies against ADAMTS13 prior to pregnancy disappeared after successful deliver. (2015)
  • Author(s): Ogawa Y, Matsumoto M, Sadakata H, Isonishi A, Kato S, Nojima Y, Fujimura Y.
  • Journal Title: Transfus Med Hemother. Volume: 42 Issue: 1 Pages: 59-63
  • DOI: 10.1159/000370225
  • Peer Reviewed / Open Access
• [Journal Article] Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension. (2015)
  • Author(s): Ohta T, Urayama K, Tada Y, Furue T, Imai S, Matsubara K, Ono H, Sakano T, Jinno K, Yoshida Y, Miyata T, Fujimura Y.
  • Journal Title: Pediatr Nephrol. Volume: 30 Issue: 4 Pages: 603-608
  • DOI: 10.1007/s00467-014-2975-4
  • Peer Reviewed / Open Access
• [Journal Article] Familial C3 glomerulonephritis associated with mutations in the gene for complement factor B. (2015)
  • Author(s): Imamura H, Konomoto T, Tanaka E, Hisano S, Yoshida Y, Fujimura Y, Miyata T, Nunoi H.
  • Journal Title: Nephrol Dial Transplant. Volume: 30 Issue: 5 Pages: 862-864
  • DOI: 10.1093/ndt/gfv054
  • Peer Reviewed / Open Access
• [Journal Article] Autoimmune-type atypical hemolytic uremic syndrome treated with eculizumab as first-line therapy. (2015)
  • Author(s): Hisano M, Ashida A, Nakano E, Suehiro M, Yoshida Y, Matsumoto M, Miyata T, Fujimura Y, Hattori M.
  • Journal Title: Pediatrics Int. Volume: 57 Issue: 2 Pages: 313-317
  • DOI: 10.1111/ped.12469
  • Peer Reviewed / Open Access
• [Journal Article] STEC:O111-HUS complicated by acute encephalopathy in a young girl was successfully treated with a set of hemodiafiltration, steroid pulse, and soluble thrombomodulin, under plasma exchange. (2015)
  • Author(s): Yada N, Fujioka M, Bennett CL, Inoki K, Miki T, Watanabe A, Yoshida T, Hayakawa M, Matsumoto M, Fujimura Y.
  • Journal Title: Clin Case Rep. Volume: 3 Issue: 4 Pages: 208-212
  • DOI: 10.1002/ccr3.196
  • Peer Reviewed / Open Access
• [Journal Article] A Novel Quantitative Hemolytic Assay Coupled with Restriction Fragment Length Polymorphisms Analysis Enabled Early Diagnosis of Atypical Hemolytic Uremic Syndrome and Identified Unique Predisposing Mutations in Japan. (2015)
  • Author(s): Yoshida Y, Miyata T, Matsumoto M, Shirotani-Ikejima H, Uchida Y, Ohyama Y, Kokubo T, Fujimura Y.
  • Journal Title: PLos One. Volume: 10 Issue: 5 Pages: e0124655-e0124655
  • DOI: 10.1371/journal.pone.0124655
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] A rapid, fully automatic and highly sensitive ADAMTS13 gold particle immunoassay using a routine biochemistry analyser. (2015)
  • Author(s): Kato S, Tanaka M, Isonishi A, Matsumoto M, Samori T, Fujimura Y.
  • Journal Title: Brit J Haematol Volume: in press Issue: 4 Pages: 655-658
  • DOI: 10.1111/bjh.13425
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Poor-responder to plasma exchange therapy in acquired TTP is associated with ADAMTS13 inhibitor boosting: Visualization of an ADAMTS13-inhibitor complex, and its proteolytic clearance from plasma. (2015)
  • Author(s): Isonishi A, Bennett C.L., Plaimauer B, Scheiflinger F, Matsumoto M, Fujimura Y.
  • Journal Title: Transfusion Volume: in press
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Candidate gene analysis using genomic quantitative PCR: identification of ADAMTS13 large deletions in two patients with Upshaw-Schulman syndrome. (2014)
  • Author(s): Eura Y, Kokame K, Takafuta T, Tanaka R, Kobayashi H, Ishida F, Hisanaga S, Matsumoto M, Fujimura Y, Miyata T.
  • Journal Title: olecular Genetics & Genomic Medicine. Volume: 2 Issue: 3 Pages: 240-244
  • DOI: 10.1002/mgg3.64
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: Concentration of C3 p.I1157T mutation. (2014)
  • Author(s): Matsumoto T, Fan X, Ishikawa E, Ito M, Amano K, Toyoda H, Komada Y, Ohishi K, Katayama N, Yoshida Y, Matsumoto M, Fujimura Y, Ikejiri M, Wada H, Miyata T.
  • Journal Title: Int J Hematol. Volume: 100 Issue: 5 Pages: 437-442
  • DOI: 10.1007/s12185-014-1655-2
  • Peer Reviewed / Open Access
• [Journal Article] Postoperative atypical hemolytic uremic syndrome associated with complement c3 mutation. (2014)
  • Author(s): Matsukuma E, Imamura A, Iwata , Takeuchi T, Yoshida Y, Fujimura Y, Fan X, Miyata T, Kuwahara T.
  • Journal Title: Case Rep Nephrol. Volume: 2014 Pages: 784943-784943
  • DOI: 10.1155/2014/784943
  • Peer Reviewed / Open Access
• [Journal Article] A first bout of thrombotic thrombocytopenic purpura triggered by herpes simplex infection in a 45-year-old nulliparous female with Upshaw-Schulman syndrome. (2014)
  • Author(s): Morioka M, Matsumoto M, Saito M, Kokame K, Miyata T, Fujimura Y.
  • Journal Title: Blood Transfus. Volume: 12
  • Peer Reviewed
• [Journal Article] Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy. (2014)
  • Author(s): Tanaka H, Tenkumo C, Mori N, Kokame K, Fujimura Y, Hata T.
  • Journal Title: J Obset Gynaecol Res. Volume: 40 Issue: 1 Pages: 247-249
  • DOI: 10.1111/jog.12125
  • Peer Reviewed
• [Journal Article] Response to 'Death during pregnancy: thrombotic thrombocytopenic purpura or septic shock?'. (2014)
  • Author(s): Takuma Y, Fujimura Y, Emoto Y, Kuriu Y, Iino M, Matoba R.
  • Journal Title: J Obstet Gynaecol Res. Volume: 40 Issue: 3 Pages: 887-887
  • DOI: 10.1111/jog.12220
  • Peer Reviewed
• [Journal Article] Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society. (2014)
  • Author(s): Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, Kaname S, Okada H, Sato W, Yasuda T, Yoshida Y, Fujimura Y, Hattori M, Kagami S.
  • Journal Title: Clin Exp Nephrol. Volume: 18 Issue: 1 Pages: 4-9
  • DOI: 10.1007/s10157-013-0911-8
  • Peer Reviewed
• [Journal Article] Effect of ADAMTS-13 on cerebrovascular microthrombosis and neuronal injury after experimental subarachnoid hemorrhage. (2014)
  • Author(s): Muroi C, Fujioka M, Mishima K, Irie K, Fujimura Y, Nakano T, Fandino J, Keller E, Iwasaki K, Fujiwara M.
  • Journal Title: J Thromb Haemost. Volume: 12(4) Issue: 4 Pages: 505-514
  • DOI: 10.1111/jth.12511
  • Peer Reviewed
• [Journal Article] Drug-induced Thrombotic Thrombocytopenic Purpura Successfully Treated with Recombinant Human Soluble Thrombomodulin (2013)
  • Author(s): Nishijima Y, Hirata H, Himeno A, Kida H, Matsumoto M, Takahashi R, Otani Y, Inoue K, Nagatomo I, Takeda Y, Kijima T, Tachibana I, Fujimura Y, Kumanogoh A.
  • Journal Title: Internal Medicine Volume: 52 Issue: 10 Pages: 1111-1114
  • DOI: 10.2169/internalmedicine.52.9180
  • NAID: 130003365599
  • ISSN: 0918-2918, 1349-7235
  • Peer Reviewed
• [Journal Article] Ratio of von Willebrand Factor Propeptide to ADAMTS13 Is Associated With Severity of Sepsis. (2013)
  • Author(s): Fukushima H, Nishio K, Asai H, Watanabe T, Seki T, Matsui H, Sugimoto M, Matsumoto M, Fujimura Y, Okuchi K.
  • Journal Title: Shock. Volume: 39 Issue: 5 Pages: 409-414
  • DOI: 10.1097/shk.0b013e3182908ea7
  • Peer Reviewed
• [Journal Article] Analysis of genetic and predisposing factors in Japanese patients with atypical hemolytic uremic syndrome. (2013)
  • Author(s): Fan X, Yoshida Y, Honda S, et al. (Matsumoto M ４番目)
  • Journal Title: Mol Immunol. Volume: 54 Issue: 2 Pages: 238-246
  • DOI: 10.1016/j.molimm.2012.12.006
  • Peer Reviewed
• [Journal Article] Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR). (2013)
  • Author(s): Bennett CL, Jacob S, Fujimura Y (last author).
  • Journal Title: Br J Haematol. Volume: 161 Issue: 6 Pages: 896-898
  • DOI: 10.1111/bjh.12303
  • Peer Reviewed
• [Journal Article] Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndorome) on hemodialysis for 19 years: a case report (2013)
  • Author(s): Mise K, Ubara Y, Matsumoto M, Sumida K, Hiramatsu R, Hasegawa E, Yamanouchi M, Hayami N, Suwabe T, Hoshino J, Sawa N, Ohashi K, Kokame K, Miyata T, Fujimura Y, Takaichi K.
  • Journal Title: BMC Nephrol. Volume: 14 Issue: 1 Pages: 156-156
  • DOI: 10.1186/1471-2369-14-156
  • Peer Reviewed
• [Journal Article] Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 2011 (2013)
  • Author(s): Kawano N, Yokota-Ikeda N, Yoshida S, Kuriyama T, Yamashita K, Sugio Y, Makino S, Ono N, Inoue Y, Himeji D, Kodama K, Uezono S, Shimao Y, Ueda A, Matsumoto M, Iino H, Fujimura Y.
  • Journal Title: Internal Medicine Volume: 52 Issue: 17 Pages: 1883-1891
  • DOI: 10.2169/internalmedicine.52.8253
  • NAID: 130003365801
  • ISSN: 0918-2918, 1349-7235
  • Peer Reviewed
• [Journal Article] Limited renal prophylaxis in regular plasmatherapy for heritable ADAMTS13 deficiency. (2013)
  • Author(s): Doi T, Ohga S, Ito N, Ishimura M, Suga N, Nomura A, Takada H, Matsumoto M, Fujimura Y, Hara T.
  • Journal Title: Pediatr Blood Cancer. Volume: 60 Issue: 9 Pages: 1557-1558
  • DOI: 10.1002/pbc.24553
  • Peer Reviewed
• [Journal Article] ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment. (2013)
  • Author(s): Hori Y, Hayakawa M, Isonishi A, Soejima K, Matsumoto M, Fujimura Y.
  • Journal Title: Transfusion. Volume: 53 Issue: 12 Pages: 3192-3202
  • DOI: 10.1111/trf.12182
  • Peer Reviewed
• [Journal Article] ADAMTS13 activity and genetic mutations in Japanese subjects. (2013)
  • Author(s): Toshiyuki Miyata, Koichi Kokame, Masanori Matsumoto, and Yoshihiro Fujimura.
  • Journal Title: Haemostaselolgie Volume: 33 Issue: 02 Pages: 131-137
  • DOI: 10.5482/hamo-12-11-0017
  • Peer Reviewed
• [Journal Article] Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry. (2013)
  • Author(s): Mansouri Taleghani M, von Krogh AS, Fujimura Y, George JN, Hrachovinová I, Knöbl PN, Quist-Paulsen P, Schneppenheim R, Lämmle B, Kremer Hovinga JA.
  • Journal Title: Hamostaseologie. Volume: 33 Issue: 02 Pages: 138-143
  • DOI: 10.5482/hamo-13-04-0026
  • Peer Reviewed
• [Journal Article] Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura. (2013)
  • Author(s): Yamamoto T, Fujimura Y, Emoto Y, Kuriu Y, Iino M, Matoba R.
  • Journal Title: J obset Gynaecol Res. Volume: 39 Issue: 1 Pages: 351-354
  • DOI: 10.1111/j.1447-0756.2012.01941.x
  • Peer Reviewed
• [Journal Article] Drug-induced Thrombotic Thrombocytopenic Purpura Successfully Treated with Recombinant Human Soluble Thrombomodulin. (2013)
  • Author(s): Nishijima Y, Hirata H, Himeno A, Kida H, Matsumoto M, Takahashi R, Otani Y, Inoue K, Nagatomo I, Takeda Y, Kijima T, Tachibana I, Fujimura Y, Kumanogoh A.
  • Journal Title: Intern Med. Volume: 52 Pages: 1111-1114
  • NAID: 130003365599
  • Peer Reviewed
• [Journal Article] Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR). (2013)
  • Author(s): Bennett CL, Jacob S, Dunn BL, et al. (Fujimura Y, last author)
  • Journal Title: Br J Haematol. Volume: 印刷中
  • Peer Reviewed
• [Journal Article] The first bout of TTP triggered by herpes simplex infection in a 45-year-old nonparous female with Upshaw-Schulman syndrome (2013)
  • Author(s): Morioka M, Matsumoto M, Saito M, Kokame K, Miyata T, Fujimura Y.
  • Journal Title: Blood Transfusion. Volume: 印刷中
  • Peer Reviewed
• [Journal Article] ADAMTS13 activity and genetic mutations in Japanese subjects (2013)
  • Author(s): Miyata T, Kokame K, Matsumoto M, Fujimura Y.
  • Journal Title: Hamostaseologie Volume: 印刷中
• [Journal Article] Therapeutic modality of 11 patients with TTP: Experience in a single institution in Miyazaki during 2000-2011 (2013)
  • Author(s): Kawano N, Yokota-Ikeda N, Sugio Y, Yoshida S, Ono N, Kuriyama T, Yamashita K, Makino S, Inoue Y, Himeji D, Kodama K, Uezono S, Shimao Y, Matsumoto M, Iino H, Fujimura Y
  • Journal Title: Intern Med. Volume: 印刷中
  • Peer Reviewed
• [Journal Article] ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: Implications for selection of plasma preparations for TTP treatment (2013)
  • Author(s): Hori Y, Hayakawa M, Isonishi A, Soejima K, Matsumoto M, Fujimura Y
  • Journal Title: Transfusion Volume: 印刷中
  • Peer Reviewed
• [Journal Article] Limited renal prophylaxis in regular plasmatherapy for heritable ADAMTS13 deficiency (2013)
  • Author(s): Doi T, Ohga S, Ito N, Ishimura M, Suga N, Nomura A, Takada H, Matsumoto M, Fujimura Y, Hara T
  • Journal Title: Pediatric Blood & Cancer Volume: 印刷中
  • Peer Reviewed
• [Journal Article] Hereditary thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) and the hereditary TTP registry (2013)
  • Author(s): Mansouri M-T,von Krogh A-S, Fujimura Y, George JN, Hrachovinova I, Knӧbl PN, Quist-Paulsen P, Schneppenheim R, Lӓmmle B, Kremer-Hovinga JA
  • Journal Title: Hamostaseologie Volume: 印刷中
  • Peer Reviewed
• [Journal Article] Pediatric idiopathic TTP diagnosed with decreased ADAMTS13 activity. (2012)
  • Author(s): Morishima T, Nomura A, Saida S, Watanabe K, Yagi H, Matsumoto M, Fujimura Y, Heike T, Nakahata T, Adachi S.
  • Journal Title: Pediatr Int. Volume: 54 Issue: 3 Pages: 422-423
  • DOI: 10.1111/j.1442-200x.2011.03467.x
  • Peer Reviewed
• [Journal Article] Identification and recombinant analysis of botrocetin-2, a snake venom cofactor for von Willebrand factor-induced platelet agglutination. (2012)
  • Author(s): Yamamoto-Suzuki Y, Sakurai Y, Fujimura Y, Matsumoto M, Hamako J, Kokubo T, Kitagawa H, Kawsar SM, Fujii Y, Ozeki Y, Matsushita F, Matsui T.
  • Journal Title: Biochemistry. Volume: 51 Pages: 5329-5338
  • Peer Reviewed
• [Journal Article] Identification of epitopes on ADAMTS13 recognized by a panel of monoclonal antibodies with functional or non-functional effects on catalytic activity. (2012)
  • Author(s): Igari A, Nakagawa T, Moriki T, Yamaguchi Y, Matsumoto M, Fujimura Y, Soejima K, Murata M.
  • Journal Title: Thromb Res. Volume: 130 Issue: 3 Pages: e79-e83
  • DOI: 10.1016/j.thromres.2012.06.006
  • Peer Reviewed
• [Journal Article] Cilostazol down-regulates the height of mural platelet thrombi formed under a high-shear rate flow in the absence of ADAMTS13 activity. (2012)
  • Author(s): Yagi H, Yamaguchi N, Shida Y, Hayakawa M, Matsumoto M, Sugimoto M, Wada H, Tsubaki K, Fujimura Y.
  • Journal Title: Eur J Pharmacol. Volume: 691 Issue: 1-3 Pages: 151-155
  • DOI: 10.1016/j.ejphar.2012.07.001
  • Peer Reviewed
• [Journal Article] Paucity of CD34-positive cells and increased expression of high-mobility group box 1 in coronary thrombus with type 2 diabetes mellitus. (2012)
  • Author(s): Yamashita A, Nishihira K, Matsuura Y, Ito T, Kawahara K, Hatakeyama K, Hashiguchi T, Maruyama I, Yagi H, Matsumoto M, Fujimura Y, Kitamura K, Shibata Y, Asada Y.
  • Journal Title: Atherosclerosis. Volume: 224 Issue: 2 Pages: 511-514
  • DOI: 10.1016/j.atherosclerosis.2012.07.027
  • Peer Reviewed
• [Journal Article] Ticlopidine-, clopidogrel-, and prasugrel-associated thrombotic thrombocytopenic purpura: a 20- year review from the Southern Network on Adverse Reactions (SONAR). (2012)
  • Author(s): Jacob S, Dunn BL, Qureshi ZP, et al. （Mastumoto M 20番目）
  • Journal Title: Semin Thromb Hemost. Volume: 38 Issue: 08 Pages: 845-853
  • DOI: 10.1055/s-0032-1328894
• [Journal Article] Two newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses (2012)
  • Author(s): Tanabe S, Yagi H, Kimura T, Isonishi A, Kato S, Yoshida Y, Hayakawa M, Matsumoto M, Ohtaki S, Takahashi Y, Fujimura Y.
  • Journal Title: Int J Hematol. Volume: 96 Issue: 6 Pages: 789-797
  • DOI: 10.1007/s12185-012-1221-8
  • NAID: 10031144620
  • Peer Reviewed
• [Journal Article] A novel Wiskott-Aldrich syndrome protein mutation in an infant with thrombotic thrombocytopenic purpura. (2012)
  • Author(s): Kawasaki Y, Toyoda H, Otsuki S, Iwasa T, Iwamoto S, Azuma E, Itoh-Habe N, Wada H, Fujimura Y, Morio T, Imai K, Mitsuiki N, Ohara O, Komada Y.
  • Journal Title: Eur J Haematol. Volume: 90 Issue: 2 Pages: 164-168
  • DOI: 10.1111/ejh.12057
  • Peer Reviewed
• [Journal Article] The ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis-in comparison with the Child-Turcotte-Pugh score and the MELD score (2012)
  • Author(s): Takaya H, Uemura M, Fujimura Y, Matsumoto M, Matsuyama T, Kato S, Morioka C, Ishizashi H, Hori Y, Fujimoto M, Tsujimoto T, Kawaratani H, Kurumatani N, FukuiH
  • Journal Title: Hepatology research Volume: 42 Issue: 5 Pages: 459-459
  • DOI: 10.1111/j.1872-034x.2011.00950.x
  • Peer Reviewed
• [Journal Article] Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency. (2012)
  • Author(s): Yagi H, Matsumoto M, Fujimura Y.
  • Journal Title: Presse Med. Volume: 41 Issue: 3 Pages: e137-e155
  • DOI: 10.1016/j.lpm.2011.10.027
• [Journal Article] Reduced larger VWF multimers at dawn in OSA plasmas reflect severity of apneic episodes (2012)
  • Author(s): Koyama N, Matsumoto M, Tamaki S, Yoshikawa M, Fujimura Y, Kimura H
  • Journal Title: Eur Resp J Volume: (印刷中) Issue: 3 Pages: 657-664
  • DOI: 10.1183/09031936.00186210
  • Peer Reviewed
• [Journal Article] The homozygous p.C1024R-ADAMTS13 gene mutation links to a late-on set phenotype of Upshaw-Schulman syndrome in Japan (2012)
  • Author(s): Taguchi F, Yagi H, Matsumoto M, Sadamura S, Isonishi A, Soejima K, Fujimura Y
  • Journal Title: Thromb Haemost Volume: (印刷中) Issue: 05 Pages: 1003-1005
  • DOI: 10.1160/th11-11-0799
  • Peer Reviewed
• [Journal Article] Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocyt openic purpura in a population from Japan (2012)
  • Author(s): Matsumoto M, Bennett CL, Isonishi A, Qureshi Z, Hori Y, Hayakawa M, Yoshida Y, Yagi H, Fujimura Y
  • Journal Title: PLoS ONE Volume: 7 Issue: 3 Pages: e33029-e33029
  • DOI: 10.1371/journal.pone.0033029
  • Peer Reviewed
• [Journal Article] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan (2011)
  • Author(s): Y.Fujimura, 他7名
  • Journal Title: J Thromb Haemost Volume: 9(review) Pages: 283-301
  • DOI: 10.1111/j.1538-7836.2011.04341.x
  • Peer Reviewed
• [Journal Article] H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS13 (2011)
  • Author(s): Akiyama R, Komori I, Hiramoto R, Isonishi A, Matsumoto M, Fujimura Y.
  • Journal Title: Internal Medicine Volume: 50 Issue: 6 Pages: 643-647
  • DOI: 10.2169/internalmedicine.50.4620
  • NAID: 130000649901
  • ISSN: 0918-2918, 1349-7235
  • Peer Reviewed
• [Journal Article] Epitope analysis of autoantibodies to ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura. (2011)
  • Author(s): Yamaguchi Y, Moriki T, Igari A, Nakagawa T, Wada H, Matsumoto M, Fujimura Y, Murata M.
  • Journal Title: Thromb Res. Volume: 128 Issue: 2 Pages: 169-173
  • DOI: 10.1016/j.thromres.2011.03.010
  • Peer Reviewed
• [Journal Article] Determination of ADAMTS13 and its clinical significance for ADAMTS13 supplementation therapy to improve the survival of patients with decompensated liver cirrhosis (2011)
  • Author(s): Uemura M, Fujimura Y, Ko S, et al.
  • Journal Title: Int J Hepatol Volume: Epub 2011 Jul 18. Pages: 1-12
  • DOI: 10.4061/2011/759047
  • Peer Reviewed
• [Presentation] TMA：最近の考え方と治療法の選択 (2015)
  • Author(s): 藤村吉博
  • Organizer: Nagoya Hematology Forum
  • Place of Presentation: ヒルトン名古屋（愛知県・名古屋市）
  • Year and Date: 2015-02-06
  • Invited
• [Presentation] Introduction of a quick assay for ADAMTS13 activity improved a survival of acquired TTP patients who received platelet transfusions. (2014)
  • Author(s): Yoshii Ｙ, Matsumoto M, Kurumatani N, Isonishi A, Uemura M, Hori Y, Hayakawa M, Yagi H, Bennett C.L., Fujimura Y
  • Organizer: The 56th Annual meeting of American Society of Hematology
  • Place of Presentation: San Francisco（USA）
  • Year and Date: 2014-12-08
• [Presentation] Decreased platelet thrombus size, due to a heightened proteolysis of VWF by ADAMTS13 is quickly restored after valve replacement in aortic stenosis patients. (2014)
  • Author(s): Yagi H, Hayakawa M, Yamaguchi N, Yamashita K, Taniguchi S, Matsumoto M, Tsubaki K, Fujimura Y
  • Organizer: The 56th Annual meeting of American Society of Hematology
  • Place of Presentation: San Francisco（USA）
  • Year and Date: 2014-12-07
• [Presentation] Registry of congenital thrombotic microangiopathy in Japan. (2014)
  • Author(s): Fujimura Y
  • Organizer: The 3rd ASEAN Federation of Hematology Congress.Plenary lecture
  • Place of Presentation: Bangkok（Thailand）
  • Year and Date: 2014-10-23
  • Invited
• [Presentation] ADAMTS13と血小板輸血-過去、現在、未来 (2014)
  • Author(s): 藤村吉博
  • Organizer: 第20回 ADAMTS13研究会
  • Place of Presentation: 奈良ホテル（奈良県・奈良市）
  • Year and Date: 2014-09-18
  • Invited
• [Presentation] TTP, HUS, aHUSの鑑別診断と治療. (2014)
  • Author(s): 藤村吉博
  • Organizer: 第59回臨床病理近畿支部例会
  • Place of Presentation: 天理医療大学キャンパス（奈良県・天理市）
  • Year and Date: 2014-06-21
  • Invited
• [Presentation] 非典型溶血性尿毒症症候群（aHUS） (2013)
  • Author(s): 藤村吉博
  • Organizer: 第75回日本血液学会学術集会
  • Place of Presentation: ロイトン札幌
  • Invited
• [Presentation] Phenotype modifiers of ADAMTS 13 deficiency with respect to gender, gene mutations, and inhibitor boosting (2012)
  • Author(s): 藤村吉博
  • Organizer: 56. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung (GTH)
  • Place of Presentation: St. Gallen (Switzerland)
• [Presentation] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan (2011)
  • Author(s): 藤村吉博
  • Organizer: XXIII Congress of the International Society on Thrombosis and Haemostasis
  • Place of Presentation: 京都国際会議場（京都府）
• [Presentation] 止血因子VWFとその特異的切断酵素ADAMTS13の均衡破綻による血栓症のパラダイムシフト (2011)
  • Author(s): 藤村吉博
  • Organizer: 奈良県医師会第一回生涯教育講座(招待講演)
  • Place of Presentation: 奈良県医師会館（奈良県）
• [Presentation] TMAとDIC：血液専門医が認識すべき病態と治療のパラダイムシフト (2011)
  • Author(s): 藤村吉博
  • Organizer: 第２回サイト研究会 (招待講演)
  • Place of Presentation: ホテル阪急インターナショナル前 エコルテホール（大阪府）
• [Presentation] ADAMTS13とFactor Hに焦点を当てた血栓性微小血管障害症(TMA)の診断と治療 (2011)
  • Author(s): 藤村吉博
  • Organizer: 第１５回 炎症と凝固研究会 (招待講演)
  • Place of Presentation: 三重大学医学部（三重県）
• [Presentation] 周産期TMA（血栓性血小板減少性紫斑病）の診断と治療 (2011)
  • Author(s): 藤村吉博
  • Organizer: 平成２３年度富山県産科婦人科学会 第４回例 (招待講演)
  • Place of Presentation: ANAクラウンプラザホテル３階飛鳥の間（富山県）
• [Presentation] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan (2011)
  • Author(s): 藤村吉博
  • Organizer: 第７３回日本血液学会学術集会（JSH2011）
  • Place of Presentation: 名古屋国際会議場（名古屋県）
• [Presentation] 後天性TTPに対する血漿交換療法
  • Author(s): 藤村吉博
  • Organizer: 第61回日本輸血・細胞治療学会総会
  • Place of Presentation: パシフィコ横浜
  • Invited
• [Presentation] Thrombotic thrombocytopenic purpura(TTP) と Hemolytic uremic syndrome(HUS) ～病態解明と診断のための新たなマーカー～
  • Author(s): 藤村吉博
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形国際ホテル
  • Invited
• [Presentation] 非典型溶血性尿毒症症候群 (aHUS): 診断と治療の進歩
  • Author(s): 藤村吉博
  • Organizer: 第35回日本血栓止血学会学術集会
  • Place of Presentation: 山形国際ホテル
  • Invited
• [Presentation] A long-term phenotype analysis of 51 patients with Upshaw -Schulman Syndrome in Japan, with special references to pregnancy and renal failure that requires hemodialysis
  • Author(s): Fujimura Y, Yoshii Y, Matsumoto M, Isonishi A, Hayakawa M, Yoshida Y, Yagi H, Kokame K, Miyata T.
  • Organizer: The 55th Annual meeting of American Society of Hematology
  • Place of Presentation: Ernest N. Morial Convention Center（New Orleans, USA)
• [Presentation] 均一な集団と考えられるADAMTS13 活性著減（＜0.5%）後天性特発性TTP 150 例の解析.
  • Author(s): 松本雅則，Bennett Charles，石西綾美，Qureshi Zenia，堀勇二，早川正樹，吉田瑶子，八木秀男，藤村吉博.
  • Organizer: 第34回日本血栓止血学会学術集会.
  • Place of Presentation: ハイアットリージェンシー（東京）
• [Presentation] TMA：診断と治療の新しい展開
  • Author(s): 藤村吉博
  • Organizer: 第21回 群馬血液疾患研究会
  • Place of Presentation: 群馬ロイヤルホテル（群馬）
  • Invited
• [Presentation] ADAMTS13-VWFの均衡破綻による血栓性微小血管障害症
  • Author(s): 藤村吉博
  • Organizer: 第３回 Global and New Insight into HSCT研究会
  • Place of Presentation: ウエスティン都ホテル京都（京都）
  • Invited
• [Presentation] 本邦でのaHUS患者のdiagnostic pathwayと登録状況
  • Author(s): 藤村吉博
  • Organizer: 第49回補体シンポジウム
  • Place of Presentation: 大阪府立成人病センター（大阪）
  • Invited
• [Presentation] TMA:診断と治療の新しい展開
  • Author(s): 藤村吉博
  • Organizer: 第19回岐阜血液研究会
  • Place of Presentation: 岐阜グランドホテル（岐阜）
  • Invited
• [Presentation] Establishing a TTP diagnostic network in Japan -Experience at Nara-
  • Author(s): Yoshihiro Fujimura
  • Organizer: International and ANZ Steering Committee for APMAT Research Network
  • Place of Presentation: Melbourne Convention Center（オーストラリア）
  • Invited
• [Presentation] 本邦におけるTMA解析状況
  • Author(s): 藤村吉博
  • Organizer: 第７回日本血栓止血学会学術標準化委員会シンポジウム
  • Place of Presentation: 東医健保会館（東京）
  • Invited
• [Presentation] TMA病態に対する新鮮凍結血漿由来ADAMTS13の治療効果
  • Author(s): 藤村吉博
  • Organizer: 石川県合同輸血療法委員会
  • Place of Presentation: 石川県地場産業振興センター（石川）
  • Invited
• [Presentation] Unusually large VWF multimers appearing in patient plasmas of HSCT predispose TMA:Management with plasma ADAMTS13 and recombinant soluble thrombomodulin
  • Author(s): Hayakawa M, Matsumoto M, Fujimura Y
  • Organizer: Jahrestagung der Gesellschaft fur Thrombose- und Hamostaseforschung (GTH)
  • Place of Presentation: ICM-Internationales Congress Center Munchen（ドイツ）
• [Presentation] ADAMTS13: Biology and Upshaw-Schulman Syndrome
  • Author(s): Yoshihiro Fujimura
  • Organizer: 23rd Annual Hemophilia Research Study Update Meeting(HRSU)
  • Place of Presentation: THE GRAND HOTEL MALAHIDE（アイルランド）
  • Invited
• [Book] ADAMTS13 book (2015)
  • Author(s): Fujimura Y, Kokame K, Yagi H, Isonishi A, Matsumoto M, Miyata T
  • Publisher: Springer (edited by George Rodgers)
• [Book] Annual Review 血液 (2013)
  • Author(s): 松本雅則、藤村吉博
  • Total Pages: 8
  • Publisher: 中外医学社
• [Book] 新版日本輸血・細胞治療学会認定医制度指定カリキュラム (2012)
  • Author(s): 藤村吉博
  • Total Pages: 3
  • Publisher: 日本輸血・細胞治療学会
• [Remarks] 難病情報センター「血栓性血小板減少性紫斑病（TTP）」
  • URL: http://www.nanbyou.or.jp/entry/87