Prophylactic fresh frozen plasma may prevent development of hepatic VOD after stem cell transplantation via ADAMTS13-mediated restoration of von Willebrand factor plasma levels

As we previously described, plasma infusion increased platelet count (PC) in four patients with IIB von Willebrand disease with severe thrombocytopenia. In a sixty years old patient in the same family, with chronic thrombocytopenia (PC = 30 000/ml) associated to an absence of large von Willebrand Factor multi-mers (vWF) in plasma, we successfully treated :1° A gastrointestinal bleeding episode with fresh frozen plasma infusion (15ml/Kg/day).2° Three months later a severe epistaxis with cryoprecipi-tate supernatant (15ml/Kg/day).During these bleeding episodes, the efficiency of these two treatments on the PC could be ascertained according to the following figureWe observed after ten days of these two treatments the following biological effects : a normalisation of vWF cross immunoelectrophoresis, of ristocetin induced normal platelet aggregation by patient's plasma, and of patient's plasma vWF binding to control platelets.In conclusion a factor appears to be present in both fresh frozen plasma and cryoprecipitate supernatant which prevents the abnormal binding of von Willebrand Factor (in this IIB von Willebrand disease) to the patient's platelets.

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Effects of fresh-frozen plasma and its cryosupernatant fraction on von Willebrand factor multimeric forms in chronic relapsing thrombotic thrombocytopenic purpura

Blood ◽

10.1182/blood.v65.5.1232.1232 ◽

1985 ◽

Vol 65 (5) ◽

pp. 1232-1236 ◽

Cited By ~ 79

Author(s):

JL Moake ◽

JJ Byrnes ◽

JH Troll ◽

CK Rudy ◽

SL Hong ◽

...

Keyword(s):

Plasma Exchange ◽

Fresh Frozen Plasma ◽

Plasma Samples ◽

Fresh Frozen ◽

Normal Human ◽

Von Willebrand ◽

Willebrand Factor ◽

Frozen Plasma

Abstract Remission plasma samples of some patients with chronic relapsing thrombotic thrombocytopenic purpura (TTP) contain unusually large von Willebrand factor (vWF) multimers similar to those produced by normal human endothelial cells in culture. The infusion of the cryosupernatant fraction of normal plasma is as effective as normal fresh-frozen plasma (FFP) in the treatment or prevention of TTP episodes in patients with the chronic relapsing form of TTP. Three patients with chronic relapsing TTP during remission have unusually large vWF multimers present in their plasma. Two of the patients were transfused once with FFP, one of the two received cryosupernatant on three occasions, and the third patient was studied before and immediately after plasma exchange. Unusually large vWF multimers decreased or disappeared from patient plasma samples within 1/2 to 1 1/2 hours following the transfusion of FFP (on two occasions) or cryosupernatant (on two of three occasions), and immediately after plasma exchange (on one occasion). The patient who received cryosupernatant was studied serially after the infusions. Unusually large vWF multimers returned to her plasma within ten to 24 hours and persisted thereafter. Unusually large vWF multimers did not disappear from patient remission plasma samples, or from the culture medium removed from normal human endothelial cells, when these fluids were incubated in vitro with either normal FFP or cryosupernatant. We conclude that an activity in FFP, and its cryosupernatant fraction, promoted the rapid in vivo disappearance of unusually large vWF multimers from the plasma of two patients with chronic relapsing TTP in remission, and plasma exchange reversed the abnormality in a third patient who was in partial remission. Neither FFP nor cryosupernatant directly converted unusually large multimers to smaller vWF forms in vitro in the fluid phase. These results indicate that an activity in the cryosupernatant fraction of normal plasma is involved in vivo in controlling the metabolism of unusually large vWF multimers, and that this process is defective in some chronic relapsing TTP patients.

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VOLUME PLASMA DAN FAKTOR VIII DALAM KRIOPRESIPITAT

INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY ◽

10.24293/ijcpml.v19i1.396 ◽

2016 ◽

Vol 19 (1) ◽

pp. 9

Author(s):

Dian Widyaningrum ◽

Purwanto AP ◽

Julia Setyati

Keyword(s):

Factor Viii ◽

Blood Group ◽

Von Willebrand Factor ◽

Plasma Volume ◽

Fresh Frozen Plasma ◽

Factor Viii Level ◽

Viii Level ◽

Fresh Frozen ◽

Von Willebrand ◽

Willebrand Factor

Blood product such as cryoprecipitate required a quality control. This includes development, implementation and the standard operating procedures use of each step of the process in the production of cryoprecipitated substance to ensure that the produced product contains a minimum of 80 international units (IU) of factor VIII. Cryoprecipitation is prepared from fresh frozen plasma that thawed and centrifuge by immediate spinning the excess plasma which then removed and leaving approximately 40ml which deposit 10 mL cryoprecipitate. One unit of cryoprecipitate contain 70–80 IU/unit factor VIII, ≥100 mg/unit von Willebrand factor, fibrinogen 5–10 mg/dL. The levels of factor VIII and von Willebrand factor (VWF) lowered in individuals with blood group O compared to individuals groups with non-O blood. This research is aimed to investigate whether plasma volume are correlated with the levels of factor VIII in cryoprecipitation. In this study purposive sampling is done in which 25 bags of cryoprecipitate materials (was storage for 11 months) from all types of blood group which were taken from storage, thawed, weighed and the plasma volume measured. Factor VIII was measured by coagulometric method. The researcher used Spearman correlation test to analyze the product, with significance degree p<0.05 and confidence interval 95%. In this study it is found plasma volume which was not related to the factor VIII level in cryoprecipitattion substance (p=0.585). Mean plasma volume of the cryoprecipitated matter was 56 mL, mean factor VIII was 83.3UI. Highest factor VIII level was 160.6 UI of cryoprecipitated blood group AB and lowest factor VIII level was 21.3 UI of cryoprecipitated blood group A.

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Coagulation profile of human COVID-19 convalescent plasma

Scientific Reports ◽

10.1038/s41598-021-04670-1 ◽

2022 ◽

Vol 12 (1) ◽

Author(s):

Allan M. Klompas ◽

Noud van Helmond ◽

Justin E. Juskewitch ◽

Rajiv K. Pruthi ◽

Matthew A. Sexton ◽

...

Keyword(s):

Coagulation Factor ◽

Protein S ◽

Coagulation Factors ◽

Fresh Frozen Plasma ◽

Thrombin Time ◽

Fresh Frozen ◽

Von Willebrand ◽

Willebrand Factor ◽

Plasmin Inhibitor ◽

Frozen Plasma

AbstractConvalescent plasma is used to treat COVID-19. There are theoretical concerns about the impact of pro-coagulant factors in convalescent plasma on the coagulation cascade particularly among patients with severe COVID-19. The aim of this study was to evaluate the coagulation profile of COVID-19 convalescent plasma. Clotting times and coagulation factor assays were compared between fresh frozen plasma, COVID-19 convalescent plasma, and pathogen-reduced COVID-19 convalescent plasma. Measurements included prothrombin time, activated partial thromboplastin time, thrombin time, fibrinogen, D-dimer, von Willebrand factor activity, von Willebrand factor antigen, coagulation factors II, V, VII–XII, protein S activity, protein C antigen, and alpha-2 plasmin inhibitor. Clotting times and coagulation factor assays were not different between COVID-19 convalescent plasma and fresh frozen plasma, except for protein C antigen. When compared to fresh frozen plasma and regular convalescent plasma, pathogen reduction treatment increased activated partial thromboplastin time and thrombin time, while reducing fibrinogen, coagulation factor II, V, VIII, IX, X, XI, XII, protein S activity, and alpha-2 plasmin inhibitor. The coagulation profiles of human COVID-19 convalescent plasma and standard fresh frozen plasma are not different. Pathogen reduced COVID-19 convalescent plasma is associated with reduction of coagulation factors and a slight prolongation of coagulation times, as anticipated. A key limitation of the study is that the COVID-19 disease course of the convalesced donors was not characterized.

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