Surgery in haemophilia patients with inhibitors, with special emphasis on orthopaedics: Madrid experience.

We report on a series of 92 surgical procedures (90 patients). It includes 35 orthopaedic procedures (33 patients) and 57 non-orthopaedic procedures (57 patients). The orthopaedic procedures include 27 radiosynovectomies (minor surgery) and eight major orthopaedic procedures. The non-orthopaedic procedures include 52 minor interventions and five major procedures. The average age of patients was 34 years (range: 8-56), and the average follow-up time was 3 years (range: 1-6). Of the 92 surgical procedures, 42 were performed with activated prothrombin complex concentrates [factor eight inhibitor bypassing agent (FEIBA)] and 47 with recombinant-activated factor VIIa (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark). Regarding FEIBA treatment in minor surgery, the initial dose was 100 IU kg(-1). After 6 h, we continued with 50 IU kg(-1) every 12 h for at least 4 days (radiosynovectomies). In minor non-orthopaedic procedures, the dose was continued until day 14. In patients who underwent surgery with the haemostatic control achieved by means of rFVIIa, the initial dose of rFVIIa in minor procedures (both orthopaedic and non-orthopaedic) was 90-120 microg kg(-1). In postoperative days 1-5, the dose was 2-4 x 90-120 microg kg(-1) q3-6 h for 24 h. In major procedures (both orthopaedic and non-orthopaedic), the dose was 120 microg kg(-1) pre-operatively, 120 microg kg(-1) q 3 h day 2/day 3-5, and then 90-120 microg kg(-1) q 6 h until day 14. There were 87 good results, four fair results and one poor result. Our study has shown that haemophilic patients with inhibitors requiring surgery can undergo orthopaedic and non-orthopaedic procedures with a high expectation of success. In other words, surgery (orthopaedic and non-orthopaedic) is now possible in haemophilia patients with inhibitors, leading to an improved quality of life for these patients.

[Evaluation of the polyethyleneglycol antiglobulin test in the detection and identification of erythrocyte antibodies]. / Valoración de la prueba de antiglobulina con polietilenglicol en la detección e identificación de anticuerpos eritrocitarios.

PURPOSE: The polyethylene glycol antiglobulin test has been found to enhance the reactivity of most alloantibodies. MATERIAL AND METHODS: To investigate the utility of polyethylene glycol antiglobulin test for detection and identification of red blood cell antibodies, a comparison study of polyethylene glycol (PEG), a low-ionic-strength additive solution (LISS) and bovine serum albumin (BSA) was conducted. The sera of 47 different patients with positive antibody screening test by the LISS method, were tested in parallel with reagent antibody-detection cells using PEG, LISS and BSA. RESULTS: In the sera of 47 patients, 57 antibodies were detected. We identified 39 antibodies by the three methods. Twelve antibodies reacted by the BSA method and the LISS method but did not react with the PEG method (8 anti-I, 1 anti-P1, 1 anti-Lea(a), and two antibodies missed by the PEG method because they did not react with anti-IgG: 1 anti-M and 1 anti-K). Three antibodies reacted only with the LISS method (3 anti-I). Four clinically significant antibodies were detected only by the PEG method (2 anti-Jka, 1 anti-Jkb, 1 anti-c). The serum from a patient with delayed hemolytic transfusion reaction and no antibody detectable by the LISS and the BSA methods was tested by the PEG method. We were able to detect an anti-Jka by PEG in the pretransfusion sample. In 24 (60%) of 40 samples with clinically significant antibodies, PEG antiglobulin reactions were stronger (total score 221) than LISS antiglobulin reactions (total score 170) and BSA antiglobulin reaction (total score 184); in 14 (35%) of 40 samples, they were identical, and in 2 (5%) agglutination in the PEG method was weaker. CONCLUSION: In our experience, the polyethylene glycol antiglobulin test is more sensitive than LISS and BSA in detecting clinically significant antibodies and is an acceptable technique for routine compatibility test.