Plasma exchange and human factor VIII concentrate in managing haemophilia A with factor VIII inhibitors.

Plasma fractionation using polyelectrolytes (PEs) has potential advantages which include simplicity of procedure, high product purity, increased yield and removal of hepatitis viruses. The purpose of this study was to assess the in-vivo response to PE-fractionated human factor VIII concentrate (PE VIII) given to 3 volunteers with severe haemophilia A. PE VIII fractionated from bulk cryo- precipitate had a high specific activity (6.74 u/mg protein), no detectable isoagglutinins, a low fibrinogen content, high ratio of VIII coagulant activity (VIII:C)to VIII-related antigen (VIIIR:Ag), and a ratio of VIII:C to VIII coagulant antigen (VIII:CAg) of approximate unity. Single infusions, each of about 2000 i.u.(25 - 40 i.u./kg), were administered i.v. over 10 mins. There were no clinical, haematological or biochemical adverse effects during the 48 hr. post-infusion observation period. Immediate post-infusion recoveries of VIII:C were 119, 102 and 70 per cent with late phase half disappearance times of 17, 19 and 17 hours respectively. These values were similar to those obtained after infusion of intermediate-purity factor VIII concentrate to the same patients, and are in accord with the results of previous studies using conventional concentrates. PE VIII has potential as a therapeutic material for patients with haemophilia A.

Download Full-text

Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand's disease

British Journal of Haematology ◽

10.1111/j.1365-2141.1982.tb03888.x ◽

1982 ◽

Vol 52 (2) ◽

pp. 259-267 ◽

Cited By ~ 124

Author(s):

E. G. D. Tuddenham ◽

R. S. Lane ◽

F. Rotblat ◽

A. J. Johnson ◽

T. J. Snape ◽

...

Keyword(s):

Factor Viii ◽

Human Factor ◽

Haemophilia A ◽

Von Willebrand's Disease ◽

Von Willebrand’S Disease ◽

Human Factor Viii

Download Full-text

Combined Factor VIII And Plasma Exchange Therapy In The Management Of Patients With Factor VIII Inhibitors

10.1055/s-0038-1652977 ◽

1981 ◽

Author(s):

R T Wensley ◽

R F Stevens ◽

A M Burn

Keyword(s):

Factor Viii ◽

Clinical Response ◽

Plasma Exchange ◽

Anticoagulant Activity ◽

Response To Therapy ◽

Response To Treatment ◽

High Dose ◽

Haemophilia A ◽

Human Factor Viii ◽

Plasma Factor

Five patients with Haemophilia A and Factor VIII inhibitors and three patients with spontaneous Factor VIII inhibitors presented with severe or life-threatening haemorrhage. All, except one, received intermittent high dose human Factor VIII therapy combined with one or more plasma exchanges. The exception was a patient with spontaneous Factor VIII inhibitors who was plasma-exchanged without receiving Factor VIII. The five Haemophilia A patients showed a uniformly good clinical response to treatment with complete resolution of their bleeding episodes. Their inhibitors were reduced to a level approaching zero and adequate plasma Factor VIII levels were achieved. In contrast, the three patients with spontaneous Factor VIII inhibitors failed to show any clinical response to therapy. They had measurable plasma Factor VIII levels before the Factor VIII therapy, but the administered Factor VIII produced no additional increment. Their inhibitor levels were only minimally altered by therapy. Studies of the inhibitors from the haemophiliacs showed complete Factor VIII neutralisation in incubation mixtures, but inhibitor plasmas from the spontaneous cases failed to completely neutralise the admixed Factor VIII. It is postulated that in haemophiliacs with antibodies, replacement therapy is associated with the formation of stable imnune complexes which remain in the intravascular space and are removed at subsequent plasma exchange. These complexes do not show coagulant or anticoagulant activity. However, in non-haemophiliacs with acquired Factor VIII inhibitors, weaker association of Factor VIII and antibody in the immune complexes may account for the measurable plasma Factor VIII activity, and also enable the dissociated antibody to diffuse out of the intravascular space and hence be unavailable for removal by plasma exchange.

Download Full-text