platelet storage pool deficiency platelet storage pool deficiency platelet storage pool deficiency is a type of coagulopathy characterized by defects in the granules in platelets, particularly a lack of granular non-metabolic adenosine diphosphate. individuals with adenosine diphosphate deficient storage pool disease present a prolonged bleeding time due to impaired aggregation response to fibrillar collagen. platelet storage pool deficiencyother namesstorage pool platelet diseaseplatelet storage pool deficiency is inherited in an autosomal dominant mannerspecialtyhematology symptomsanemiacausesinherited or acquireddiagnostic methodflow cytometry, bleeding time analysistreatmentantifibrinolytic medications symptoms and signs anemia the presentation (signs/symptoms) of an individual with platelet storage pool deficiency is as follows: unusual bleeding(after surgical procedure) anemia decrease mean platelet volume myelodysplasia cause hairy cell leukemia the condition of platelet storage pool deficiency can be acquired or inherited (genetically passed on from the individuals parents). some of the causes of platelet storage pool deficiency when acquired are: hairy-cell leukemia cardiovascular bypass mechanism platelet structure in terms of the pathophysiology of platelet storage pool deficiency one must consider several factors including the human body's normal function prior to such a deficiency, such as platelet alpha-granules one of three types of platelet secretory granule platelet α–granules are important in platelet activity, α–granules connect with plasma membrane. this in turn increases the size of the platelet. platelet α–granules have an important role in hemostasis as well as thrombosis. snare accessory proteins control the secretion of α–granule. diagnosis on for example optical densitometry, a first and second wave of platelet aggregation is seen, in this case for an adp-initiated aggregation. the second wave is absent in platelet storage pool deficiency. the diagnosis of this condition can be done via the following: flow cytometry bleeding time analysis platelet aggregation function study: platelet aggregation function by disorders and agonists adpepinephrinecollagenristocetin p2y receptor defect (including clopidogrel) decreasednormalnormalnormal adrenergic receptor defect normaldecreasednormalnormal collagen receptor defect normalnormaldecreased or absentnormal von willebrand disease bernard–soulier syndrome normalnormalnormaldecreased or absent glanzmann's thrombasthenia afibrinogenemia decreaseddecreaseddecreasednormal or decreased storage pool deficiency absent second wavepartial aspirin or aspirin-like disorder absent second waveabsentnormal types this condition may involve the alpha granules or the dense granules. therefore, the following examples include: flow cytometry analysis platelet alpha-granules gray platelet syndrome quebec platelet disorder dense granules δ-storage pool deficiency hermansky–pudlak syndrome chédiak–higashi syndrome treatment platelet storage pool deficiency has no treatment however management consists of antifibrinolytic medications if the individual has unusual bleeding event, additionally caution should be taken with usage of nsaids