Based on the results of our pilot study, extensive clinical studies are warranted to investigate the role of gabapentin in the management of symptomatic LSS.

Desmopressin is an analog of vasopressin that exerts a substantial haemostatic effect by inducing the release of von Willebrand factor from its storage sites in endothelial cells. It has proved useful in treating or preventing bleeding episodes in patients with von Willebrand disease, haemophilia A and platelet function defects. Its efficacy in achieving a satisfactory level of haemostasis has reduced the use of blood products to treat bleeding episodes. Clinicians need to become familiar with the use of this drug that has become a home medication for many patients with inherited bleeding disorders.

Rare bleeding disorders (RBDs) comprise 3-5% of all congenital bleeding disorders. They can evade typical coagulation screening tests and there is a poor correlation between laboratory results and bleeding phenotype. Thromboelastography (TEG) measures coagulation globally in whole blood samples. The aims of this study were to evaluate the utility of TEG as an adjunct to the routine screening tests employed for the diagnosis of RBDs and to correlate TEG results with the bleeding phenotype in RBDs. TEG parameters and clot kinetics were compared to bleeding phenotypes (asymptomatic, mild, moderate and severe) in 26 RBD patients and 30 normal controls. Clot kinetics correlated strongly with RBDs and with the severity of bleeding phenotype with mean maximum rate of thrombus generation (MRTG) 15.4 mm min(-1) in controls vs. 6.0 in RBDs (P < 0.0001, Wilcoxin). The mean MRTG was 7.7 in mildly symptomatic, 5.5 in moderately symptomatic and 4.1 in severely symptomatic patients (P < 0.0001, Kruskal-Wallis). Disorders that are often missed by conventional screening tests, dysfibrinogenaemia and platelet disorders displayed a distinctive TEG curve with markedly decreased maximum amplitude (MA) and low MRTG values. Factor XIII and PAI deficient patients displayed increased fibrinolysis in addition to low MRTGs. All patients with RBDs, but none of the normal controls, had abnormal clot kinetics suggesting that TEG may be an effective screening test for RBDs.

Because of the increase in the use of warfarin in the population in recent years, reversal of warfarin-related coagulopathy has become common in daily hospital practice. Transfusion of fresh frozen plasma (FFP) is the preferred treatment method for urgent warfarin reversal in the USA. We have undertaken a 1-month audit of FFP usage to ascertain the impact of warfarin use on the consumption of FFP. Sixty percent of the 376 units of FFP that were transfused during the study month were used to reverse warfarin effects. The most common reason to reverse warfarin was bleeding. Thirty-three percent of the units were used for the treatment of other coagulopathies, 7% were used in therapeutic plasmapheresis, and <1% was transfused empirically. One hundred and eighteen patients received FFP during the study month. The study population consisted mostly of elderly patients (65%); however, the warfarin reversing patients consisted disproportionately more of elderly patients (75%) compared with patients receiving FFP for other reasons (46%) (P 5 0.0032). Warfarin reversal emerged as the major indication for FFP use in this study. Blood banks of hospitals serving a predominantly elderly patient population should anticipate a higher consumption of FFP. Careful monitoring of warfarin therapy, stringent implementation of the warfarin reversal guidelines, and the introduction of newer products for warfarin reversal would help reduce the consumption of FFP. Am. J. Hematol. 82:1091Hematol. 82: -1094Hematol. 82: , 2007