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1. Thrombotic thrombocytopenic purpura: 100 years of research on Moschcowitz syndrome

Author

Cataland, Spero R., Coppo, Paul, Scully, Marie, Lämmle, Bernhard, Alberio, Lorenzo, Antun, Ana, Ay, Cihan, Agosti, Pasquale, Azoulay, Elie, Baker, Ross, Benhamou, Ygal, Boechat, Tiago, Brinkkötter, Paul, Chaturvedi, Shruti, Crawley, James, De Cristofaro, Raimondo, Garma, Julio del Río, Dutt, Tina, De Groot, Rens, Rubia, Javier de la, Falter, Tanja, Farias, João, Friedman, Kenneth, Fujimura, Yoshihiro, Gavriilaki, Eleni, George, James N., Graça, Nuno A. G., Hassenpflug, Wolf-Achim, Izquierdo, Cristina Pascual, Joly-Laffargue, Bérangère, Kentouche, Karim, Knoebl, Paul, Kokame, Koichi, Hovinga, Johanna Kremer, Kühne, Lucas, Kyrle, Paul, Lester, Will, Mancini, Ilaria, Masias, Camila, Matsumoto, Masanori, Mazepa, Marshall, Miesbach, Wolfgang, Metjian, Ara, Mingot-Castellano, Maria-Eva, Miyata, Toshiyuki, Moake, Joel, Muia, Joshua, Patriquin, Chris, Pavenski, Katerina, Prohaszka, Zoltan, Peyvandi, Flora, Reti, Marienn, Rossmann, Heidi, Sakai, Kazuya, Sarode, Ravi, Schneppenheim, Reinhard, Schraner, Marissa, Singh, Deepak, Sinkovits, György, Stubbs, Matthew, Studt, Jan-Dirk, Sukumar, Senthil, Thomas, Mari, Tóth, Andor, Vanhoorelbeke, Karen, Veyradier, Agnes, Völker, Linus, Sophie von Krogh, Anne, Voorberg, Jan, Waage, Anders, Westwood, JP, Wood, Erica, Yagi, Hideo, and Zheng, X. Long

Abstract

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Published
2024
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2. Thrombotic thrombocytopenic purpura: 100 years of research on Moschcowitz syndrome

Author

Alberio, Lorenzo, Antun, Ana, Ay, Cihan, Agosti, Pasquale, Azoulay, Elie, Baker, Ross, Benhamou, Ygal, Boechat, Tiago, Brinkkötter, Paul, Chaturvedi, Shruti, Crawley, James, De Cristofaro, Raimondo, Garma, Julio del Río, Dutt, Tina, De Groot, Rens, Rubia, Javier de la, Falter, Tanja, Farias, João, Friedman, Kenneth, Fujimura, Yoshihiro, Gavriilaki, Eleni, George, James N., Graça, Nuno A. G., Hassenpflug, Wolf-Achim, Izquierdo, Cristina Pascual, Joly-Laffargue, Bérangère, Kentouche, Karim, Knoebl, Paul, Kokame, Koichi, Hovinga, Johanna Kremer, Kühne, Lucas, Kyrle, Paul, Lester, Will, Mancini, Ilaria, Masias, Camila, Matsumoto, Masanori, Mazepa, Marshall, Miesbach, Wolfgang, Metjian, Ara, Mingot-Castellano, Maria-Eva, Miyata, Toshiyuki, Moake, Joel, Muia, Joshua, Patriquin, Chris, Pavenski, Katerina, Prohaszka, Zoltan, Peyvandi, Flora, Reti, Marienn, Rossmann, Heidi, Sakai, Kazuya, Sarode, Ravi, Schneppenheim, Reinhard, Schraner, Marissa, Singh, Deepak, Sinkovits, György, Stubbs, Matthew, Studt, Jan-Dirk, Sukumar, Senthil, Thomas, Mari, Tóth, Andor, Vanhoorelbeke, Karen, Veyradier, Agnes, Völker, Linus, Sophie von Krogh, Anne, Voorberg, Jan, Waage, Anders, Westwood, JP, Wood, Erica, Yagi, Hideo, Zheng, X. Long, Cataland, Spero R., Coppo, Paul, Scully, Marie, and Lämmle, Bernhard

Published
2024
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6. Genotype-Phenotype Correlation in Congenital TTP: New Insights from a Multicentre Study with 121 Patients

Author

Van Dorland, Anette Anette, primary, Mansouri Taleghani, Magnus, additional, Friedman, Kenneth Dale, additional, George, James, additional, Hrachovinova, Ingrid, additional, Knöbl, Paul, additional, Lammle, Bernhard, additional, Matsumoto, Masanori, additional, von Krogh, Anne Sophie, additional, Schneppenheim, Reinhard, additional, Aebi-Huber, Isabella, additional, Lukas, Bütikofer, additional, Cermakova, Zuzana, additional, Quist-Paulsen, Petter, additional, Terrell, Deirdra, additional, Vesely, Sara K., additional, Fujimura, Yoshihiro, additional, and Kremer Hovinga Strebel, Johanna A., additional

Published
2018
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10. Quantification of the Actual Numbers of Transplantable CD34+CD133+ Hematopoietic Stem Cells Residing in the Umbilical Cord Blood (UCB) Units: A New Indicator of Quality Assurance of UCB Units

Author

Matsuoka, Yoshikazu, primary, Fujioka, Tatsuya, additional, Sumide, Keisuke, additional, Hatanaka, Kazuo, additional, Nakamura, Fumiaki, additional, Matsumoto, Kayoko, additional, Otani, Satoshi, additional, Kimura, Takafumi, additional, Fujimura, Yoshihiro, additional, Asano, Hiroaki, additional, and Sonoda, Yoshiaki, additional

Published
2016
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11. Investigator-Initiated Phase 2 Trial of Rituximab in Adult Japanese Patients with Treatment-Resistant Acquired Thrombotic Thrombocytopenic Purpura

Author

Miyakawa, Yoshitaka, primary, Imada, Kazunori, additional, Ichinohe, Tatsuo, additional, Yamane, Yusuke, additional, Nishio, Kenji, additional, Abe, Takayuki, additional, Fujimura, Yoshihiro, additional, Ueda, Yasunori, additional, Matsumoto, Masanori, additional, and Okamoto, Shinichiro, additional

Published
2015
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19. Deficient ADAMTS13 Activity in Ticlopidine-Associated Thrombotic Thrombocytopenic Purpura (TTP) in Populations From Japan in 2012: Validation of Findings Initially Reported in the United States in 1998 and 2000.

Author

Bennett, Charles, primary, Matsumoto, Masanori, additional, Georgantopoulos, Peter, additional, Jacob, Sony, additional, Dunn, Brianne L, additional, Qureshi, Zaina Parvez, additional, Bandarenko, Nicholas, additional, Kwaan, Hau C., additional, Winters, Jeffrey, additional, Sarode, Ravindra, additional, Kiss, Joseph E, additional, Danielson, Constance, additional, Ortel, Thomas L., additional, Raife, Thomas, additional, Clark, William F, additional, Rock, Gail, additional, Zheng, X. Long, additional, Raisch, Dennis, additional, and Fujimura, Yoshihiro, additional

Published
2012
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27. Potential Role of Enhanced Cytokinemia and Endotoxemia on the Decreased Activity of Plasma ADAMTS13 in Patients with Alcoholic Hepatitis.

Author

Ishikawa, Masatoshi, primary, Uemura, Masahito, primary, Matsuyama, Tomomi, primary, Matsumoto, Masanori, primary, Fujimoto, Masao, primary, Ishizashi, Hiromichi, primary, Kato, Seiji, primary, Morioka, Chie, primary, Tsujimoto, Tatsuhiro, primary, Kitazawa, Toshiyuki, primary, Kawaratani, Hidehito, primary, Fujimura, Yoshihiro, primary, and Fukui, Hiroshi, primary

Published
2008
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29. Decreased Activity of Plasma ADAMTS13 Parallels Enhanced Endotoxemia in Patients with Severe Acute Pancreatitis: Relationship to Multiorgan Failure

Author

Morioka, Chie, primary, Uemura, Masahito, primary, Matsuyama, Tomomi, primary, Matsumoto, Masanori, primary, Fujimoto, Masao, primary, Kato, Seiji, primary, Ishikawa, Masatoshi, primary, Ishizashi, Hiromichi, primary, Tsujimoto, Tatsuhiro, primary, Kawaratani, Hideto, primary, Okuchi, Kazuo, primary, Nishio, Kenji, primary, Fujimura, Yoshihiro, primary, and Fukui, Hiroshi, primary

Published
2008
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31. Plasma ADAMTS13 Activity Markedly Decreases in Patients with Severe Acute Pancreatitis: Its Potential Role on the Development of Multiorgan Failure.

Author

Morioka, Chie, primary, Uemura, Masahito, additional, Matsuyama, Tomomi, additional, Matsumoto, Masanori, additional, Kato, Seiji, additional, Ishikawa, Masatohsi, additional, Ishizashi, Hiromichi, additional, Fujimoto, Masao, additional, Tsujimoto, Tatsuhiro, additional, Nishio, Kenji, additional, Okuchi, Kazuo, additional, Fujimura, Yoshihiro, additional, and Fukui, Hiroshi, additional

Published
2007
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32. Clinical and Outcomes Findings for Thrombotic Thrombocytopenic Purpura among 467 Persons with Severely Versus Not Severely Deficient ADAMTS-13 Levels.

Author

Bennett, Charles L., primary, Luu, Thanh Ha, primary, Zakarija, Anaadriana, primary, Kwaan, Hau C., primary, Bandarenko, Nicholas, primary, Pandey, Dilip K., primary, Cursio, John F., primary, McKoy, June M., primary, Buffie, Charlie, primary, Winters, Jeffrey L., primary, Ortel, Thomas L., primary, Sarode, Ravindra, primary, Zheng, X. Long, primary, Raife, Thomas J., primary, Matsumoto, Masanori, primary, Fujimura, Yoshihiro, primary, and Rock, Gail A., primary

Published
2007
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33. Natural History of 33 Patients with Upshaw-Schulman Syndrome Has Revealed That All the Gravida Develop Thrombocytopenia, Often Followed by Thrombotic Microangiopathy with Stillbirth.

Author

Fujimura, Yoshihiro, primary, Matsumoto, Masanori, additional, Kokame, Koichi, additional, Yagi, Hideo, additional, Isonishi, Ayami, additional, Matsuyama, Tomomi, additional, Kato, Seiji, additional, Ishizashi, Hiromichi, additional, Shida, Yasuaki, additional, Kenji, Nishio, additional, Akiyama, Nobu, additional, Tomiyama, Junji, additional, Natori, Kazuhiro, additional, Kuraishi, Yasunobu, additional, Imamura, Yutaka, additional, Inoue, Nobumasa, additional, Higasa, Satoshi, additional, Seike, Masako, additional, Kozuka, Teruhiko, additional, Hara, Masamichi, additional, Sugimoto, Mitsuhiko, additional, Wada, Hideo, additional, Murata, Mitsuru, additional, Miyata, Toshiyuki, additional, and Ikeda, Yasuo, additional

Published
2007
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34. Ticlopidine- and Clopidogrel-Associated Thrombotic Thrombocytopenic Purpura (TTP): Final Results from the Surveillance Epidemiology and Risk Factor-TTP Study Group.

Author

Richey, Elizabeth A., primary, Bennett, Charles L., primary, Kwaan, Hau C., primary, Zakarija, Anaadriana, primary, Banderanko, Nicholas, primary, Pandey, Dilip K., primary, McKoy, June M., primary, Yarnold, Paul R., primary, Raisch, Dennis W., primary, Winters, Jeffrey L., primary, Raife, Thomas J., primary, Cursio, John F., primary, Fisher, Matthew J., primary, Ortel, Thomas L., primary, Tallman, Martin S., primary, Matsumoto, Masanori, primary, Fujimura, Yoshihiro, primary, and Moake, Joel L., primary

Published
2007
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35. Upshaw-Schulman Syndrome Revealed by Pregnancy.

Author

Seike, Masako, primary, Kozuka, Teruhiko, primary, Kokame, Koichi, primary, Miyata, Toshiyuki, primary, Matsuyama, Tomomi, primary, Matsumoto, Masanori, primary, Fujimura, Yoshihiro, primary, and Hara, Masamichi, primary

Published
2006
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36. Infusions of Fresh Frozen Plasma to the Patients with a High-Risk Group for Hepatic VOD Associated with Stem Cell Transplantation Reduce Its Occurrence.

Author

Matsumoto, Masanori, primary, Kawa, Keisei, additional, Yagi, Hieo, additional, Park, Yong-Dong, additional, Takeshima, Yasuhiro, additional, Kosaka, Yoshiyuki, additional, Hara, Hiroshi, additional, Kai, Shunro, additional, Yamamoto, Masuji, additional, Kanemaru, Akihisa, additional, Fukuhara, Shirou, additional, Hino, Masayuki, additional, Sako, Masahiko, additional, Hiraoka, Akira, additional, Ogawa, Hiroyasu, additional, Hara, Junichi, additional, and Fujimura, Yoshihiro, additional

Published
2006
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37. Increased Plasma VWF Antigen over Decreased ADAMTS13 Activity in Patients with Liver Cirrhosis: Relationship to Degree of Liver Disturbance and Clinical Features.

Author

Uemura, Masahito, primary, Kato, Seiji, additional, Matsumoto, Masanori, additional, Ishizashi, Hiromichi, additional, Isonishi, Ayami, additional, Hiura, Hisahide, additional, Matsuyama, Tomomi, additional, Yagi, Hideo, additional, Ishikawa, Masatoshi, additional, Fujimoto, Masao, additional, Kojima, Hideyuki, additional, Yoshiji, Hitoshi, additional, Fujimura, Yoshihiro, additional, and Fukui, Hiroshi, additional

Published
2006
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39. Upshaw-Schulman Syndrome: A Masqueraded Thrombocytopenia during Pregnancy.

Author

Matsuyama, Tomomi, primary, Matsumoto, Masanori, additional, Kato, Seiji, additional, Akiyama, Nobu, additional, Tomiyama, Junji, additional, Natori, Kazuhiko, additional, Kuraishi, Yasunobu, additional, Imamura, Yutaka, additional, Inoue, Nobumasa, additional, Kokame, Koichi, additional, Miyata, Toshiyuki, additional, and Fujimura, Yoshihiro, additional

Published
2005
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40. Localization of ADAMTS13 to the stellate cells of human liver

Author

Uemura, Masahito, primary, Tatsumi, Kouko, additional, Matsumoto, Masanori, additional, Fujimoto, Masao, additional, Matsuyama, Tomomi, additional, Ishikawa, Masatoshi, additional, Iwamoto, Taka-aki, additional, Mori, Toshio, additional, Wanaka, Akio, additional, Fukui, Hiroshi, additional, and Fujimura, Yoshihiro, additional

Published
2005
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47. Molecular characterization of ADAMTS13gene mutations in Japanese patients with Upshaw-Schulman syndrome

Author

Matsumoto, Masanori, Kokame, Koichi, Soejima, Kenji, Miura, Masayoshi, Hayashi, Syuhei, Fujii, Yasuhiko, Iwai, Asayuki, Ito, Etsuro, Tsuji, Yoichiro, Takeda-Shitaka, Mayuko, Iwadate, Mitsuo, Umeyama, Hideaki, Yagi, Hideo, Ishizashi, Hiromichi, Banno, Fumiaki, Nakagaki, Tomohiro, Miyata, Toshiyuki, and Fujimura, Yoshihiro

Abstract

We report here 7 new mutations in the ADAMTS13gene responsible for Upshaw-Schulman syndrome (USS), a catastrophic phenotype of congenital thrombotic thrombocytopenic purpura, by analyzing 5 Japanese families. There were 3 mutations that occurred at exon-intron boundaries: 414+1G>A at intron 4, 686+1G>A at intron 6, and 1244+2T>G at intron 10 (numbered from the A of the initiation Met codon), and we confirmed that 2 of these mutations produced aberrantly spliced messenger RNAs (mRNAs). The remaining 4 mutations were missense mutations: R193W, I673F, C908Y, and R1123C. In expression experiments using HeLa cells, all mutants showed no or a marginal secretion of ADAMTS13. Taken together with the findings in our recent report we determined the responsible mutations in a total of 7 Japanese patients with USS with a uniform clinical picture of severe neonatal hyperbilirubinemia, and in their family members, based on ADAMTS13gene analysis. Of these patients, 2 were homozygotes and 5 were compound heterozygotes. The parents of one homozygote were related (cousins), while those of the other were not. Molecular models of the metalloprotease, fifth domain of thrombospondin 1 (Tsp1-5), and Tsp1-8 domains of ADAMTS13 suggest that the missense mutations could cause structural defects in the mutants.

Published
2004
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48. SCID-repopulating cell activity of human cord blood–derived CD34−cells assured by intra–bone marrow injection

Author

Wang, Jianfeng, Kimura, Takafumi, Asada, Rumiko, Harada, Sachio, Yokota, Shouhei, Kawamoto, Yoshio, Fujimura, Yoshihiro, Tsuji, Takashi, Ikehara, Susumu, and Sonoda, Yoshiaki

Abstract

Precise analysis of human CD34-negative (CD34−) hematopoietic stem cells (HSCs) has been hindered by the lack of a simple and reliable assay system of these rare cells. Here, we successfully identify human cord blood–derived CD34−severe combined immunodeficiency (SCID)– repopulating cells (SRCs) with extensive lymphoid and myeloid repopulating ability using the intra–bone marrow injection (IBMI) technique. Lineage-negative (Lin−) CD34−cells did not show SRC activity by conventional tail-vein injection, possibly due to their low levels of homing receptor expression and poor SDF-1/CXCR4– mediated homing abilities, while they clearly showed a high SRC activity by IBMI. They generated CD34+progenies not only in the injected left tibia but also in other bones following migration. Moreover, they showed slower differentiating and reconstituting kinetics than CD34+cells in vivo. These in vivo–generated CD34+cells showed a distinct SRC activity after secondary transplantation, clearly indicating the long-term human cell repopulating capacity of our identified CD34−SRCs in nonobese diabetic (NOD)/SCID mice. The unveiling of this novel class of primitive human CD34−SRCs by IBMI will provide a new concept of the hierarchy in the human HSC compartment and has important implications for clinical HSC transplantation as well as for basic research of HSC.

Published
2003
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49. SCID-repopulating cell activity of human cord blood–derived CD34− cells assured by intra–bone marrow injection

Author

Wang, Jianfeng, Kimura, Takafumi, Asada, Rumiko, Harada, Sachio, Yokota, Shouhei, Kawamoto, Yoshio, Fujimura, Yoshihiro, Tsuji, Takashi, Ikehara, Susumu, and Sonoda, Yoshiaki

Abstract

Precise analysis of human CD34-negative (CD34−) hematopoietic stem cells (HSCs) has been hindered by the lack of a simple and reliable assay system of these rare cells. Here, we successfully identify human cord blood–derived CD34− severe combined immunodeficiency (SCID)– repopulating cells (SRCs) with extensive lymphoid and myeloid repopulating ability using the intra–bone marrow injection (IBMI) technique. Lineage-negative (Lin−) CD34− cells did not show SRC activity by conventional tail-vein injection, possibly due to their low levels of homing receptor expression and poor SDF-1/CXCR4– mediated homing abilities, while they clearly showed a high SRC activity by IBMI. They generated CD34+ progenies not only in the injected left tibia but also in other bones following migration. Moreover, they showed slower differentiating and reconstituting kinetics than CD34+cells in vivo. These in vivo–generated CD34+ cells showed a distinct SRC activity after secondary transplantation, clearly indicating the long-term human cell repopulating capacity of our identified CD34− SRCs in nonobese diabetic (NOD)/SCID mice. The unveiling of this novel class of primitive human CD34− SRCs by IBMI will provide a new concept of the hierarchy in the human HSC compartment and has important implications for clinical HSC transplantation as well as for basic research of HSC.

Published
2003
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50. The von Willebrand Factor Domain–Mediating Botrocetin-Induced Binding to Glycoprotein IB Lies Between Val449and Lys728

Author

Fujimura, Yoshihiro, Holland, Linda Z., Ruggeri, Zaverio M., and Zimmerman, Theodore S.

Abstract

Botrocetin, a component of Bothrops jararacavenom, induces von Willebrand factor (vWF)-dependent platelet agglutination and has been proposed as an alternative agent to ristocetin for evaluating vWF function. However, important differences between the vWF-platelet interactions induced by these two agents have suggested that different regions of vWF and the platelet may be involved in the interactions induced by the two agonists. We have recently demonstrated that binding of vWF to the platelet glycoprotein (GP) lb receptor, either induced by ristocetin or as occurs spontaneously with asialo-vWF or vWF from IIB von Willebrand disease, is mediated by a domain residing on a 52/48-kilodalton (kD) tryptic fragment of vWF. This fragment extends from amino acid residue Val (449) to Lys (728). We have now found that this 52/48-kD fragment blocks botrocetin-induced binding of vWF to platelets and completely inhibits botrocetin-induced platelet agglutination. These results provide evidence that the vWF domain-mediating, botrocetin-induced platelet agglutination lies within the region delimited by this fragment and is therefore close to or identical with that which mediates ristocetin-induced binding and spontaneous binding of vWF to platelet GPIb. Anti-GPIb monoclonal antibodies also blocked agglutination, which showed that botrocetin, like ristocetin, induces binding of vWF to the GPIb receptor.

Published
1987
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