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Coagulation Disorders Platelet Disorders
coagulation, platelet and packed red cell transfusions or even for three or four days. For GT or BSS patients in whom isoantibodies
recombinant FVIIa). The association of octeotride with or without an are suspected, the survival of transfused platelets can be assessed
oestrogen–progesterone combination should be considered in the by flow cytometry. For patients with a moderate or weak bleeding
management of recurrent GI bleeding due to angiodysplasia and has tendency, and depending on the type of surgery, local procedures
been successfully used in GT.
It should be proposed early for can be used. One option is to perfuse tranexamic acid during
patients with repeated GI bleeding.
The most difficult situation to the immediate period after surgery.
deal with is intracranial haemorrhage with the risk of lesions.
Desmopressin, commonly used for patients with vWD, is also
Preventative Treatment Prior to Surgery recommended for patients with moderate thrombocytopenia or a mild
A challenge is to predict the haemorrhagic risk for patients with platelet functional defect; while increasing VWF levels at the injured
moderate bleeding symptoms during surgery or childbirth. It is site, it may also have a direct effect on platelets.
important to know about the recent and past history of nose or gum such as WAS, Chediak–Higashi syndromes, Griscelli syndromes or
bleeding, easy bruising, the presence of menorraghia or excessive AML, require specialist treatment and haematopoietic stem cell
bleeding during previous surgery or childbirth. Not only is the transplanation at an early age.
intensity of the bleeding episodes important, but also their frequency;
haemoglobin and ferritin levels can reflect severity. The notion of a Conclusions
bleeding score is useful. This should take into account a patient’s age, Growing evidence suggests that inherited diseases of platelets are more
as bleeding frequency often decreases with age. frequent than previously thought. Efforts are being made to distinguish
FT from ITP to avoid diagnosis after patients have received medications
For patients with repeated and severe bleeding, haemorrhagic risk for controlling an immune-mediated process. Recent progress in
is important and platelets are systematically transfused. The quantity diagnosis will not only be valuable for the patients, but will also
of normal (transfused) platelets required to avoid bleeding has continue to identify targets, such as new treatments for patients with
been assessed as 50,000/µl and this level should be maintained ischaemic disease, for controlling platelet functions clinically. ■
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20 EUROPEAN HAEMATOLOGY 2007
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