STRORAGE POOL DISEASE

• Pathological Basis:
  • Mode of inheritance: variable.
  • Patients with storage pool disease lack or have diminished amounts of dense granules, storage pool of ADP and ATP. Alpha granules may or may not be absent.
  • Storage pool disease has been described in association with a number of clinical conditions: Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, Wiskott-Aldrich syndrome, thrombocytopenia-absent radius (TAR) syndrome.
• Treatment:
  • Platelet concentrates: given judiciously to prevent the risk for platelet alloimmunization.
  • Avoid any medications that contain aspirin.