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.

## Symptoms and signs

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

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

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

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 :

| | ADP | Epinephrine | Collagen | Ristocetin |
| - | - | - | - | - |
| P2Y receptor defect (including Clopidogrel) | Decreased | Normal | Normal | Normal |
| Adrenergic receptor defect | Normal | Decreased | Normal | Normal |
| Collagen receptor defect | Normal | Normal | Decreased or absent | Normal |
| Von Willebrand disease Bernard–Soulier syndrome | Normal | Normal | Normal | Decreased or absent |
| Glanzmann's thrombasthenia Afibrinogenemia | Decreased | Decreased | Decreased | Normal or decreased |
| Storage pool deficiency | Absent second wave | Absent second wave | Absent second wave | Partial |
| Aspirin or aspirin-like disorder | Absent second wave | Absent second wave | Absent | Normal |

### Types

This condition may involve the alpha granules or the dense granules. Therefore, the following examples include:

* 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

