Management of severe VWD with cryoprecipitate collected by repeated apheresis of a single dedicated donor
Identifieur interne : 000C69 ( Istex/Checkpoint ); précédent : 000C68; suivant : 000C70Management of severe VWD with cryoprecipitate collected by repeated apheresis of a single dedicated donor
Auteurs : Gregory J. Pomper [États-Unis] ; Margaret E. Rick [États-Unis] ; Jay S. Epstein [États-Unis] ; Elizabeth J. Read [États-Unis] ; Susan F. Leitman [États-Unis]Source :
- Transfusion [ 0041-1132 ] ; 2003-11.
English descriptors
- Teeft :
- American association, Apheresis, Apheresis procedure, Autologous plasma, Blood banks, Brinogen content, Coagulation test results, Cryoprecipitate, Cryoprecipitate units, David ginsburg, Ddavp, Ddavp stimulation, Ddavpstimulated plasma exchange, Donation, Donor, Donor exposure, Excellent hemostatic function, Excessive hemorrhage, Fviii, Hemorrhagic events, Individual units, Infusion, Mcleod, Normal growth, November, Partial thromboplastin time, Plasma, Plasma collection, Plasma exchange, Plasma exchange donation, Plasmaexchange donation, Replacement dose, Single donor, Storage data, Storage intervals, Storage times, Transfusion, Transfusion volume, Treatment regimen, Willebrand, Willebrand disease, Willebrand factor.
Abstract
BACKGROUND: Rare and severe forms of VWD are associated with trace or absent VWF. The feasibility of supporting a child with severe VWD from birth through age 12 with cryoprecipitate derived from DDAVP‐stimulated plasma exchange of a single dedicated donor is reported. STUDY DESIGN AND METHODS: An infant with excessive hemorrhage at circumcision was found to have Type 3 VWD. His father carried an allele with a mutation at the level of VWF mRNA expression but did not have a history of bleeding. Cryoprecipitate was prepared from serial DDAVP‐stimulated plasma exchanges of the father. RESULTS: Repeated plasma‐exchange donations were performed to provide all of the VWF needed for his son. An average of 14 cryoprecipitate units was prepared from each donation, and the units contained markedly elevated levels of FVIII:C. The cryoprecipitate was stored for up to 102 months. Components tested after more than 8 years of storage showed 48 to 130 percent of original FVIII:C activity. Ninety‐seven percent of the bleeding episodes, such as epistaxis, tongue‐biting accidents, and other minor lacerations, were successfully managed with a single 50‐ to 100‐percent replacement dose of FVIII. The patient experienced normal growth and development and is free of any long‐term sequelae attributable to his disease. CONCLUSIONS: Cryoprecipitate prepared by repeated plasma exchange of a VWD carrier provided excellent hemostatic function, even after storage intervals of more than a year. Plasma exchange of a committed donor was a cost‐effective and safe option for long‐term management of VWD.
Url:
DOI: 10.1046/j.1537-2995.2003.00550.x
Affiliations:
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Pomper</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>From the Departments of Transfusion Medicine and Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health; and the Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda</wicri:cityArea></affiliation><affiliation wicri:level="1"><country wicri:rule="url">États-Unis</country></affiliation></author><author><name sortKey="Rick, Margaret E" sort="Rick, Margaret E" uniqKey="Rick M" first="Margaret E." last="Rick">Margaret E. Rick</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>From the Departments of Transfusion Medicine and Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health; and the Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda</wicri:cityArea></affiliation></author><author><name sortKey="Epstein, Jay S" sort="Epstein, Jay S" uniqKey="Epstein J" first="Jay S." last="Epstein">Jay S. Epstein</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>From the Departments of Transfusion Medicine and Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health; and the Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda</wicri:cityArea></affiliation></author><author><name sortKey="Read, Elizabeth J" sort="Read, Elizabeth J" uniqKey="Read E" first="Elizabeth J." last="Read">Elizabeth J. Read</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>From the Departments of Transfusion Medicine and Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health; and the Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda</wicri:cityArea></affiliation></author><author><name sortKey="Leitman, Susan F" sort="Leitman, Susan F" uniqKey="Leitman S" first="Susan F." last="Leitman">Susan F. Leitman</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>From the Departments of Transfusion Medicine and Laboratory Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health; and the Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda</wicri:cityArea></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Transfusion</title><title level="j" type="alt">TRANSFUSION</title><idno type="ISSN">0041-1132</idno><idno type="eISSN">1537-2995</idno><imprint><biblScope unit="vol">43</biblScope><biblScope unit="issue">11</biblScope><biblScope unit="page" from="1514">1514</biblScope><biblScope unit="page" to="1521">1521</biblScope><biblScope unit="page-count">8</biblScope><publisher>Blackwell Science Inc</publisher><pubPlace>Oxford, UK and Malden, USA</pubPlace><date type="published" when="2003-11">2003-11</date></imprint><idno type="ISSN">0041-1132</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0041-1132</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>American association</term><term>Apheresis</term><term>Apheresis procedure</term><term>Autologous plasma</term><term>Blood banks</term><term>Brinogen content</term><term>Coagulation test results</term><term>Cryoprecipitate</term><term>Cryoprecipitate units</term><term>David ginsburg</term><term>Ddavp</term><term>Ddavp stimulation</term><term>Ddavpstimulated plasma exchange</term><term>Donation</term><term>Donor</term><term>Donor exposure</term><term>Excellent hemostatic function</term><term>Excessive hemorrhage</term><term>Fviii</term><term>Hemorrhagic events</term><term>Individual units</term><term>Infusion</term><term>Mcleod</term><term>Normal growth</term><term>November</term><term>Partial thromboplastin time</term><term>Plasma</term><term>Plasma collection</term><term>Plasma exchange</term><term>Plasma exchange donation</term><term>Plasmaexchange donation</term><term>Replacement dose</term><term>Single donor</term><term>Storage data</term><term>Storage intervals</term><term>Storage times</term><term>Transfusion</term><term>Transfusion volume</term><term>Treatment regimen</term><term>Willebrand</term><term>Willebrand disease</term><term>Willebrand factor</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">BACKGROUND: Rare and severe forms of VWD are associated with trace or absent VWF. The feasibility of supporting a child with severe VWD from birth through age 12 with cryoprecipitate derived from DDAVP‐stimulated plasma exchange of a single dedicated donor is reported. STUDY DESIGN AND METHODS: An infant with excessive hemorrhage at circumcision was found to have Type 3 VWD. His father carried an allele with a mutation at the level of VWF mRNA expression but did not have a history of bleeding. Cryoprecipitate was prepared from serial DDAVP‐stimulated plasma exchanges of the father. RESULTS: Repeated plasma‐exchange donations were performed to provide all of the VWF needed for his son. An average of 14 cryoprecipitate units was prepared from each donation, and the units contained markedly elevated levels of FVIII:C. The cryoprecipitate was stored for up to 102 months. Components tested after more than 8 years of storage showed 48 to 130 percent of original FVIII:C activity. Ninety‐seven percent of the bleeding episodes, such as epistaxis, tongue‐biting accidents, and other minor lacerations, were successfully managed with a single 50‐ to 100‐percent replacement dose of FVIII. The patient experienced normal growth and development and is free of any long‐term sequelae attributable to his disease. CONCLUSIONS: Cryoprecipitate prepared by repeated plasma exchange of a VWD carrier provided excellent hemostatic function, even after storage intervals of more than a year. Plasma exchange of a committed donor was a cost‐effective and safe option for long‐term management of VWD.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Maryland</li></region></list><tree><country name="États-Unis"><region name="Maryland"><name sortKey="Pomper, Gregory J" sort="Pomper, Gregory J" uniqKey="Pomper G" first="Gregory J." last="Pomper">Gregory J. Pomper</name></region><name sortKey="Epstein, Jay S" sort="Epstein, Jay S" uniqKey="Epstein J" first="Jay S." last="Epstein">Jay S. Epstein</name><name sortKey="Leitman, Susan F" sort="Leitman, Susan F" uniqKey="Leitman S" first="Susan F." last="Leitman">Susan F. Leitman</name><name sortKey="Pomper, Gregory J" sort="Pomper, Gregory J" uniqKey="Pomper G" first="Gregory J." last="Pomper">Gregory J. Pomper</name><name sortKey="Read, Elizabeth J" sort="Read, Elizabeth J" uniqKey="Read E" first="Elizabeth J." last="Read">Elizabeth J. Read</name><name sortKey="Rick, Margaret E" sort="Rick, Margaret E" uniqKey="Rick M" first="Margaret E." last="Rick">Margaret E. Rick</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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