Presence of HLA antibodies in single-donor-derived fresh frozen plasma compared with pooled, solvent detergent-treated plasma (OctaplasR)

ZusammenfassungDas Smith-Lemli-Opitz-Syndrom wird durch einen Defekt des letzten Schrittes der Cholesterolbiosynthese, den Mangel an 7-Dehydrocholesterolreduktase, verursacht. Die Akkumulation der Metaboliten 7-Dehydrocholesterol und 8-Dehydrocholesterol, die die wichtigsten biochemischen Marker für die Diagnose der Erkrankung darstellen, sowie der Mangel an Cholesterol können zu multiplen kongenitalen Anomalien führen. Die Ursache des Enzymmangels sind Mutationen innerhalb des DHCR7-Gens, welches auf Chromosom 11q13 lokalisiert ist. Therapeutische Möglichkeiten bestehen in der Gabe von Cholesterol und im Notfall Fresh Frozen Plasma (FFP); der therapeutische Nutzen von Statinen befindet sich zurzeit in der klinischen Erprobung.

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The Recovery of Factor VIII from Fresh-Frozen, Indated and Outdated Human Plasma

Thrombosis and Haemostasis ◽

10.1055/s-0038-1648010 ◽

1976 ◽

Vol 36 (01) ◽

pp. 071-077 ◽

Cited By ~ 2

Author(s):

Daniel E. Whitman ◽

Mary Ellen Switzer ◽

Patrick A. McKee

Keyword(s):

Factor Viii ◽

Polyacrylamide Gel Electrophoresis ◽

Sodium Dodecyl ◽

The United States ◽

Fresh Frozen Plasma ◽

Red Cross ◽

Random Samples ◽

Fresh Frozen ◽

Factor Viii Concentrates ◽

Frozen Plasma

SummaryThe availability of factor VIII concentrates is frequently a limitation in the management of classical hemophilia. Such concentrates are prepared from fresh or fresh-frozen plasma. A significant volume of plasma in the United States becomes “indated”, i. e., in contact with red blood cells for 24 hours at 4°, and is therefore not used to prepare factor VIII concentrates. To evaluate this possible resource, partially purified factor VIII was prepared from random samples of fresh-frozen, indated and outdated plasma. The yield of factor VIII protein and procoagulant activity from indated plasma was about the same as that from fresh-frozen plasma. The yield from outdated plasma was substantially less. After further purification, factor VIII from the three sources gave a single subunit band when reduced and analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. These results indicate that the approximately 287,000 liters of indated plasma processed annually by the American National Red Cross (ANRC) could be used to prepare factor VIII concentrates of good quality. This resource alone could quadruple the supply of factor VIII available for therapy.

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Haemophilia in a Female

Thrombosis and Haemostasis ◽

10.1055/s-0038-1653669 ◽

1971 ◽

Vol 26 (02) ◽

pp. 205-210

Author(s):

J. A McBride ◽

J Hunter ◽

Elizabeth Pearse ◽

Yvette Sultan ◽

J. P Caen

Keyword(s):

Fresh Frozen Plasma ◽

Plasma Fibrinogen ◽

Fresh Frozen ◽

Frozen Plasma

SummaryA case of haemophilia in a female is described together with the response of the patient’s level of antihaemophilic factor in the plasma following transfusion of fresh frozen plasma, fibrinogen and cryoprecipitate.

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