Background (PFDs)
- Heterogeneous group of conditions affecting the function of platelets in primary haemostasis
- Inherited PFDs may be caused by specific genetic mutations e.g. Glanzmann's Thrombasthenia or Bernard Soulier Syndrome
- More often, the genetic cause of the PFD is not known and the conditions are described as Non-Specific PFDs
- Likely to present with mucocutaneous bleeding including recurrent epistaxis, easy bruising, excessive bleeding after dental extraction or surgery, menorrhagia and post-partum haemorrhage in women
- There are a variety of treatment options for platelet function disorders
- The patient's CCC must be contacted to determine the optimal treatment for each patient and clinical scenario
Severity
- Severity of the bleeding disorder is variable
- Check prior bleding (and response to Rx) Hx
- The patient's CCC will be able to advise on the bleeding severity for individual patients
Treatment options
Prescribers must ensure that they prescribe the correct treatment. Treatment options include the use of the following:
- Random Donor Platelets
- Human Leukocyte Antigen (HLA) Matched Platelets
- Recombinant Factor VIIa
- DDAVP
- Tranexamic Acid (TXA)
The prescriber must note that not all patients with PFDs require platelet transfusion and the use of alternative treatments may be indicated e.g. DDAVP or Tranexamic Acid.
The patient's treatment of choice must be confirmed with the relevant CCC.
Platelet Transfusion
- Where the CCC directs that the use of HLA matched platelets is indicated they must be ordered from the Irish Blood Transfusion Service
- More at National treatment guidelines (CUH Intranet)
Recombinant Factor VIIa/NovoSeven
- Details at National treatment guidelines (CUH Intranet)
DDAVP/ Desmopressin
- Details at National treatment guidelines (CUH Intranet)
TXA
- Details at National treatment guidelines (CUh Intranet)
- Caution should be used when administering Tranexamic acid to patients receiving FEIBA or recombinant factor VIIa (risk of thrombosis)