Platelet storage pool disorder, disease
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(delta-storage pool disease)
Quantitative or qualitative deficiency in dense secretory or delta granules contained in the platelets. These granules contain ADP, ATP, serotonin, Ca ++, Mg, histamine and pyrophosphates. The release of these granules plays an important role in the primary hemostasis (platelet or white thrombus formation). The clinical manifestations are highly variable: easy bruising, epistaxis, gingivorragies, profuse menstruation but also bleeding after childbirth, tooth extraction or surgery.
Different aspects:
- Chediak-Higashi syndrome
- Hermansky-Pudlak syndrome
- Jacobsen syndrome
- Wiskott-Aldrich Syndrome
- TAR syndrome: radial aplasia and thrombocytopenia
- some hematological malignancies
- following allogeneic bone marrow transplantation
- some cases of rheumatoid polyarthritis and systemic lupus erythematosus
- terminal chronic renal failure
- after cardiopulmonary bypass
The diagnosis of the thrombopathy is difficult. Tests with ristocetin and electron microscopy examination.
There is no curative treatment: in case of bleeding, transfusion of platelets, trying to avoid alloimmunization (preferably single donor). In some cases: desmopressin (Minirin®) 0.3 µg/kg IV in 30 minutes, 60 minutes before a potentially hemorrhagic procedure is helpful.
Anesthetic implications:
check primary hemostasis; avoid aspirin; although it does not measure the interaction of platelets with the vessel wall, thromboelastography is useful to evaluate the primary hemostasis.
References :
Updated: October 2018