Surgery--a challenge in haemophiliacs with inhibitors.
Treatment of haemophiliacs with inhibitors is of great concern in low-income countries confronting shortage in substitutive treatment. Invasive interventions on these patients represent a major challenge due to the fact that costs are significantly higher in comparison to similar procedures conducted on patients without inhibitors.


OBJECTIVE
In the context of insufficient availability of clotting factor, we aimed at highlighting the experience of surgical treatment in inhibitor patients. We analyzed the indications, types of performed interventions and outcomes.


METHODS
This single center, retrospective analysis has been conducted on 7 inhibitor patients registered and treated in Haemophilia Center of Timisoara over ten years (1997-2007): six patients with severe hemophilia A (3 - high titer, 3 - low titer), one patient with von Willebrand disease (low titer).Three patients developed inhibitors only after 2-5 days post surgery.


RESULTS
A total of 15 invasive procedures were carried out: 2 orthopedic interventions (1 arthrodesis, 1 arthroscopic synovectomy), 2 urogenital interventions (1 surgical testicular detorsion, 1 orchiectomy), 4 limb amputations (2 bilateral upper and 2 lower limb amputation), 2 pseudotumour (PT) surgery interventions, 5 drainages (2 massive pyohaemothorax, 1 drainage of shank haematoma, 1 drainage of compressive forearm haematoma, 1 drainage of thigh haematoma). Haemostasis was achieved in patients with low level inhibitors (< 5 BU/ml) with high doses of FVIII concentrates; in those with high inhibitor level (> 5 BU/ml), surgery was managed using by-passing agents. Supplementation with local fibrin glue and intravenous or local antifibrinolytic agents was given in 68.75% of interventions. Postoperative complications consisted of haemorrhagic shock in 13.33% of interventions and infection in 6.66%. Haemostatic outcome was evaluated by blood loss and duration of treatment, compared to expectations for non-inhibitor patients. The outcome was excellent and good in 66.66% of interventions, and fair in 33.33%. Discussion, conclusion: Indication of invasive procedures in haemophiliacs with inhibitors was limited to life and/or limb-threatening situations. In low-income countries, inhibitor and recovery of FVIII monitoring is mandatory in the postoperative follow-up of patients with low or no substitution prior to surgery due to false negative results at the preoperative investigation.
Serban
M
M
Victor Babes University of Medicine and Pharmacy, Timisoara, Romania. mserban@spitalcopiitm.ro
Poenaru
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Pop
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Ionita
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Mihailov
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MD
Tepeneu
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Bădeţi
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Lighezan
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Schramm
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W
D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003029:Coagulants / Q000008:administration & dosage; D017707:Erythrocyte Transfusion; D005169:Factor VIII / Q000008:administration & dosage; D015942:Factor VIIa / Q000008:administration & dosage; D017048:Health Care Costs; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy / Q000628:therapy; D006801:Humans; D019106:Postoperative Hemorrhage / Q000517:prevention & control; D011994:Recombinant Proteins / Q000008:administration & dosage; D012189:Retrospective Studies; D012383:Romania; D013514:Surgical Procedures, Operative / Q000191:economics; D016896:Treatment Outcome; D055815:Young Adult; D014842:von Willebrand Diseases / Q000150:complications* / Q000188:drug therapy / Q000628:therapy
D001779:Blood Coagulation Factors; D003029:Coagulants; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa