The Role of Intensive Plasma Exchange in the Prevention and Management of Haemorrhage in Patients with Inhibitors to Factor VIII

Abstract

A strategy for the prevention and management of haemorrhage in patients with inhibitors to factor VIII by intensive plasma exchange and human factor VIII infusion is described. The advantages and disadvantages of this approach are discussed in the light of the authors' experience with the technique and the alternative methods of treatment described in the literature.

Treatment of patients with acquired inhibitors

“…Later the same group found evidence for a quantitative trait locus in F12 which influenced both FXII levels and thrombosis risk [9]. A previously reported polymorphism in the 5¢-untranslated region of the F12 gene (46 CfiT) [10], of which the T-allele is associated with reduced plasma FXII levels [10,11], explained part of the linkage signal [9]. In a subsequent study Tirado et al reported a 3-fold increased risk of venous thrombosis for carriers of the 46TT genotype (crude OR 3.1; 95% CI 1.1-8.7) and concluded that the 46T-allele is an independent genetic risk factor for venous thrombosis in the Spanish population [12].…”

“…In central nervous system and other life-threatening bleeding events, NovoSeven is recommended as initial therapy, with the caveat that no head-to-head data are available comparing NovoSeven and FEIBA (comparative trials are under way). Exchange plasmapheresis and/or extracorporeal immunoadsorption should be considered in patients with high antibody levels (100 BU) who do not demonstrate a clinical response to bypassing agents [11][12][13][14]. The group noted, however, that these therapies are not readily available to most physicians.…”

Plasma Exchange in Autoimmune Thrombocytopenic Purpura

“…Plasma exchange was carried out using an Aminco continuous flow cell separator (Celltrifuge) as pre viously described [9], Venous access was gained with Butterfly needles and all patients were heparinized with 1,500-2,500 1U supplemented during the procedure by a continuous infusion. Up to 4-litre procedures were carried out where possible, and replacement fluid was with plasma protein fraction, Haemaccel and normal saline containing calcium and potassium supplements.…”

Acquired Factor VIII Inhibitor in a Non-Haemophilic Patient: Successful Treatment with Plasma Exchange Associated with Factor VIII Concentrate and Immunosuppressors

“…In a recent review of 215 cases treated with steroids and cyclophosphamide or aza thioprine, 21 % fo the patients died in spite of treatment [1], Plasmapheresis has been used in combination with immunodepressants by Pintado et al [7], and in combination with factor VIII concentrates by Slocombe et al [8], More recently, Sultan et al [9] employed plasma exchange in combination with pred nisone and factor VIII concentrates. It seems that a sustained remission may be obtained by removing the inhibitor with plasma ex change and restoring normal factor VIII lev els with concentrate administration.…”