Literature DB >> 8060470

Transplantation of spleen cells in patients with hemophilia A. A report of 20 cases.

L Liu1, S Xia, J Seifert.   

Abstract

It has been reported that coagulation factor VIII (F. VIII) is produced in the spleen and other organs. Transplantation of splenic whole organ and spleen cells may, therefore, be used to treat patients with hemophilia A. The donor spleen from brain-dead donors was used to prepare spleen cell suspension for transplantation. Twenty-two spleen cell transplantations were performed on 20 patients suffering from severe hemophilia A at our institutes. Two of them underwent a second infusion of spleen cells since there was no increase in plasma F. VIII activity after the first transplantation. All but two patients showed a marked clinical improvement. Increased plasma F. VIII activity was observed in 18 of 20 cases. The peak plasma F. VIII activity in these recipients rose to 10%-15% posttransplantation in 14 cases and to over 15% in 4 cases from pretransplant levels of 0%-3%. Generally, the elevation of plasma F. VIII activity could be detected 4-7 days following transplantation of spleen cells and this lasted from 22 to 58 weeks. Four patients whose peak plasma F. VIII activity was greater than 15% experienced an uneventful course after transplantation. The patients with plasma F. VIII activity over 10% showed less frequent bleeding and prolonged intervals between bleed as well as improvement in hemophilic arthrosis. Two patients who had interval hematuria before transplantation did not have any relapse for up to 2 years after infusion of the spleen cells. These results indicate that spleen cell transplantation may be a promising method for the management of patients with hemophilia A.

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Year:  1994        PMID: 8060470     DOI: 10.1007/bf00327088

Source DB:  PubMed          Journal:  Transpl Int        ISSN: 0934-0874            Impact factor:   3.782


  7 in total

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2.  Role of bone marrow transplantation for correcting hemophilia A in mice.

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4.  FVIII expression by its native promoter sustains long-term correction avoiding immune response in hemophilic mice.

Authors:  Simone Merlin; Rosella Famà; Ester Borroni; Diego Zanolini; Valentina Bruscaggin; Silvia Zucchelli; Antonia Follenzi
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5.  A conditional knockout mouse model reveals endothelial cells as the principal and possibly exclusive source of plasma factor VIII.

Authors:  Scot A Fahs; Matthew T Hille; Qizhen Shi; Hartmut Weiler; Robert R Montgomery
Journal:  Blood       Date:  2014-04-04       Impact factor: 22.113

6.  Extrahepatic sources of factor VIII potentially contribute to the coagulation cascade correcting the bleeding phenotype of mice with hemophilia A.

Authors:  Diego Zanolini; Simone Merlin; Maria Feola; Gabriella Ranaldo; Angela Amoruso; Gianluca Gaidano; Mauro Zaffaroni; Alessandro Ferrero; Sandra Brunelleschi; Guido Valente; Sanjeev Gupta; Maria Prat; Antonia Follenzi
Journal:  Haematologica       Date:  2015-04-24       Impact factor: 9.941

7.  Characterization and visualization of murine coagulation factor VIII-producing cells in vivo.

Authors:  Morisada Hayakawa; Asuka Sakata; Hiroko Hayakawa; Hikari Matsumoto; Takafumi Hiramoto; Yuji Kashiwakura; Nemekhbayar Baatartsogt; Noriyoshi Fukushima; Yoichi Sakata; Katsue Suzuki-Inoue; Tsukasa Ohmori
Journal:  Sci Rep       Date:  2021-07-21       Impact factor: 4.379

  7 in total

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