Practical aspects of factor concentrate use in patients with von Willebrand disease undergoing invasive procedures: a European survey.

INTRODUCTION: The bleeding propensity in von Willebrand disease (VWD) is usually moderate or mild and patients with VWD do not need continuous treatment, but do require extra increased haemostatic cover when undergoing dental or surgical procedures. Desmopressin can be effective in certain patient groups and this has been considered in a previous publication. AIM: This paper now seeks to evaluate current knowledge and practice in the use of factor concentrate in the management of VWD patients undergoing invasive procedures. METHODS: A literature search was performed on the use of factor concentrates to cover invasive procedures and a survey of current practice in a number of specialist haematology centres across Europe represented by the European Haemophilia Strategy Board was conducted. RESULTS: Our review of the literature and the results of the survey showed considerable heterogeneity in treatment regimens, and a lack of consistency in reporting of the variables that determine factor concentrate dosing and monitoring. CONCLUSION: By analysing the literature, examining guidelines and using consensus deliberation, this survey allowed the group to develop recommendations for management of VWD patients undergoing invasive procedures.

Practical aspects of DDAVP use in patients with von Willebrand Disease undergoing invasive procedures: a European survey.

INTRODUCTION: Desamino D-arginine vasopressin (DDAVP or desmopressin) is a useful and effective haemostatic treatment for patients with von Willebrand Disease (VWD). However, there are still issues regarding in which subtypes of VWD DDAVP is appropriate and little consensus on its use in different surgical settings. We also lack information concerning the appropriate laboratory parameters that should be monitored. AIM: The European Haemophilia Therapy Strategy Board (EHTSB) wished to investigate published information and clinical use of DDAVP in VWD patients. METHODS: We conducted a literature survey on management of VWD during surgical interventions and undertook a survey of specialist haematologist centres across Europe to assess current management of VWD patients. RESULTS: DDAVP is ineffective in type 3 VWD and its use in type 2B remains controversial due to the possibility of thrombocytopenia. It can, however, be used effectively to cover minor surgery and dental procedures in most other VWD patients. For major surgery there is wider use of factor concentrate in preference to DDAVP depending on the subtype of VWD. We give consensus recommendations on the use of DDAVP for surgical interventions in VWD including laboratory parameters that denote an adequate response and contraindications to its use. CONCLUSIONS: DDAVP can be recommended to cover invasive procedure in selected patients with VWD, however, we need more information and systematic recording of adverse events associated with DDAVP use in VWD. A companion paper will be published covering the use of factor concentrates in VWD patients.

Oral and general health-related quality of life among young patients with haemophilia.

The clinical diagnosis of dental diseases may indicate their cause and prognosis, however it gives little information about resulting levels of impairment from the patients' perspective. In this study, we aimed to investigate oral and general health-related quality of life (OHRQoL-HRQoL) in patients with haemophilia; and to test whether haemophiliacs would have worse or better OHRQoL compared with the general population. Data were collected from haemophiliacs (age range 14-35; mean 23 +/- 6.58, n = 71) and age/sex-matched controls (age range 14-35; mean 21.00 +/- 6.45, n = 60) through face-to-face interviews including nine questions and using oral health impact profile (OHIP)-14, oral health-related quality of life-UK (OHQoL-UK), short-form general measure of health (SF)-36 to measure self-rating oral health status, perceived dental treatment needs, tooth brushing frequencies, OHRQoL-HRQoL. In the field of self-rating oral health status, perceived dental treatment needs, tooth brushing frequencies and OHIP, OHQoL-UK, SF-36 scores - except the subscales including vitality, role emotional and mental health - the control group is in better conditions compared with the haemophilia group. At the same time, both the two groups are in good conditions in dental attendance, vitality, role emotional and mental health. Life quality is related with the perceived discrepancy between the reality of what a person has and the concept of what that person wants, needs or expects. In order to eliminate the dilemma in the field of health, we should facilitate the haemophiliacs' lives by serving the health care in a multidisciplinary view.

Leukemic children: clinical and histopathological gingival lesions.

The relation between leukemia and gingiva was investigated in the present study. An extensive tumor infiltration in the gingiva was established in 1 of 2 patients with acute leukemia (AML). An inflammatory cell infiltration in various degrees was noticed in total 28 patients with leukemia (L). Various degrees of epithelium hyperplasia in all patients existing in 14 patients with medium intensity and occurring in 14 patients with light intensity of hyperplasia were observed. An increase in the keratinization of epithelium, in 3 patients with medium intensity and 1 patient with light intensity was found. It was also seen that there were fungal infections in 3 patients with medium intensity, in 6 patients with light intensity and existing in a total 26 patients with acute lymphoblastic leukemia (ALL).