(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:

• an isolated and congenital form with an autosomal dominant inheritance
• forms associated with other inherited diseases where thrombocytopenia is often present such as:

-        Chediak-Higashi syndrome

-        Hermansky-Pudlak syndrome

-        Jacobsen syndrome

-        Wiskott-Aldrich Syndrome

-        TAR syndrome: radial aplasia and thrombocytopenia

• a form where the deficit is associated with a deficiency in alpha type secretory granules 
• secondary forms:

-        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 : 

• Masliah-Planchon J, Flaujac C, Tapon-Bretaudière J, Cramer-Bordé EM, Darnige L. 
  Mise au point : la maladie du pool vide plaquettaire. 
  Ann Biol Clin 2008 ; 66 : 365-9.
• Rajpal G, Pomerantz JM, Ragni MV, Waters JH, Vallejo MC. 
  The use of thromboelastography for the peripartum management of a patient with platelet storage pool disorder. 
  Int J Obstetr Anesth 2011; 20: 173-7.

Updated: October 2018