Registry of inherited bleeding disorders in Poland--current status and potential role of the HemoRec database.

We present data collected in HemoRec, an Internet-based platform implemented in 2006 in 15 haemophilia treatment centres in Poland and compare them with the national registry of inherited bleeding disorders established since 1991 at the Institute of Haematology and Blood Transfusion in Warsaw. We also analyse the current status of haemophilia treatment in Poland as well as future perspectives. Data on 1102 patients registered in HemoRec were analysed and compared with 4294 patients in the national registry (status as at 17.08.2009). The number of patients with severe haemophilia, mild/moderate haemophilia and von Willebrand in HemoRec is 530, 328 and 54 (respectively), compared with 1199, 1167 and 1128 in the national registry. The mean age of all haemophilic patients registered in HemoRec is 26.2 years, compared with 37.3 years in the general Polish male population in 2008. The number of haemophilic patients with inhibitor registered in HemoRec is 102 compared with 155 in the national registry (resulting in a prevalence of 14.9% of all severe haemophilia A and 1.6% of all severe haemophilia B patients). HemoRec includes data on a representative group of Polish haemophilic patients, mostly with haemophilia and haemophilia with inhibitor. von Willebrand's disease is largely under-registered in Poland. The survival of Polish haemophilic patients is shorter than that in the general population. The number of inhibitor patients in Poland is relatively large and should be decreased by wider availability of immunotolerance induction in 2010.

Venostasis but not DDAVP infusion provokes the plasma fibronectin increase.

Plasma fibronectin (pFN), von Willebrand factor antigen (vWf:Ag), factor VIII procoagulant activity, fibrinogen, euglobulin lysis time (ELT) and hematocrit were determined in healthy blood donors before and after venostasis as well as after intravenous infusion of 1-deamino-8-D-arginine vasopressin (DDAVP). Both venostasis and DDAVP provoked an increase in vWf:Ag and shortening in the ELT. In contrast, venostasis only but not DDAVP induced an increase in pFN levels which was statistically significant with and without correction for a concomitant hematocrit increment. The results indicate that there is a distinct difference in the patterns of venostasis and DDAVP mediated release of proteins from the vessel wall.

[Comparison of platelet and leukocyte content in platelet concentrates obtained from CS-3000 and third generation (CS-3000 plus and Cobe-Spectra) cell separators]. / Porównanie zawartosci plytek krwi i leukocytów w koncentratach krwinek plytkowych, otrzymanych przy uzyciu separatora CS-3000 oraz separatorów III generacji (CS-3000 plus i Cobe-Spectra).

Platelet concentrates (PCs) obtained using old generation of cell separators contain high number of leukocytes. White cell (WBC) contamination and platelet (Plts) number in PCs obtained from separator II-nd generation CS-3000 and separators III-rd generations CS-3000 plus and Cobe-Spectra have been determined. PCs from new separators contain the same Plts number as PCs from CS-3000. The average leukocyte count in PCs obtained from CS-3000 was 171.26 x 10(6), whereas WBC number in PCs from CS-3000 plus and Cobe-Spectra were 2.87 and 2.54 x 10(6) respectively. In about 85% of PCs obtained from III-rd generation cell separators the leukocyte count did not exceed 5 x 10(6). This count is considered sufficient to prevent alloimmunization of HLA antigens. The determination of WBC count in every PCs allows to select PCs with fewer than 5 x 10(6) leukocytes and to transfuse them without the necessity of using expensive filters for leukocyte removing.

Secretory response of the vessel wall to DDAVP and venous occlusion in von Willebrand's disease.

The aim of this study was to compare the secretory response of the vascular wall in vivo to DDAVP (i.v. 0.3 microgram/kg, 30 min) and to venous occlusion (VO, 20 min) in control healthy subjects, patients with von Willebrand's disease type I (vWd I) and patients with von Willebrand's disease type III (vWd III). In controls (n = 10) and vWd I (n = 12), DDAVP induced a 2 to 3-fold rise in plasma von Willebrand factor antigen (vWf: Ag), factor VIII coagulant activity (VIII: C) and tissue--type plasminogen activator antigen (t-PA:Ag). VO was less effective in increasing vWf: Ag and VIII:C but produced a greater rise in t-PA:Ag. Large increments (over 10-fold) were observed in plasmin-alpha 2-antiplasmin complexes following both stimuli. In vWd III (n = 10), DDAVP and VO failed to increase vWf:Ag, VIII:C and t-PA:Ag. No significant changes in plasmin-alpha 2-antiplasmin complexes were observed in this group. Moreover, the baseline t-PA:Ag values were significantly lower in vWd III (2.17 +/- 1.13 ng/ml) than in controls (4.84 +/- 1.97 ng/ml, p < 0.001). A significant increase in urokinase--type plasminogen activator antigen (u-PA:Ag) was found only in controls after VO. Neither controls nor patients with vWd showed any changes in plasma fibronectin levels following DDAVP. The low t-PA:Ag results and the abnormal fibrinolytic response to DDAVP and VO in patients with severe (type III) vWd indicate that their endothelial cell abnormality is more extensive than the defect in the synthesis or release of vWf.