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Platelet Storage Pool Deficiency

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Disorder characterized by a decrease or lack of platelet dense bodies in which the releasable pool of adenine nucleotides and 5HT are normally stored.

Also Known As:

Acquired Storage Pool Disease; Deficiency, Platelet Storage Pool; Deficiency, Storage Pool; Familial Platelet Storage Pool Disease; Platelet Storage Pool Deficiencies; Platelet Storage Pool Disease; Storage Pool Deficiency, Platelet; Storage Pool Platelet Disease; Deficiencies, Storage Pool; Storage Pool Deficiencies; Storage Pool Deficiency

Networked: 121 relevant articles (3 outcomes, 9 trials/studies)

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases
- Hematologic Diseases: 1962

Related Diseases

1.	Anemia
2.	Renal Insufficiency (Renal Failure)
3.	Thrombasthenia (Glanzmann Thrombasthenia)
4.	von Willebrand Diseases (von Willebrand's Disease)
5.	Gray Platelet Syndrome

Experts

1.	White, James G: 3 articles (01/2013 - 09/2004)
2.	Gahl, W A: 3 articles (09/2007 - 03/2000)
3.	Gahl, William A: 2 articles (07/2013 - 02/2002)
4.	Lecchi, Anna: 2 articles (11/2012 - 03/2010)
5.	Cattaneo, Marco: 2 articles (11/2012 - 03/2010)
6.	White, J G: 2 articles (02/2009 - 09/2007)
7.	Huizing, Marjan: 2 articles (12/2008 - 02/2002)
8.	Muramatsu, M: 2 articles (08/2001 - 06/2001)
9.	Huizing, M: 2 articles (11/2000 - 03/2000)
10.	Anikster, Y: 2 articles (11/2000 - 03/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Platelet Storage Pool Deficiency:

1.	Serotonin (5 Hydroxytryptamine)IBA 08/01/1998 - "The FHR has an inherited platelet storage-pool deficiency and a widespread impairment of serotonin storage. " 05/15/1998 - "Correction of platelet 5-HT storage pool deficiency is not evident in this stage but 5-HT still influences complex mechanisms of primary hemostasis. " 04/01/1995 - "In patients with delta-storage pool deficiency, platelet aggregation at high shear rate and the bleeding time were significantly correlated with the platelet serotonin content. " 05/01/1994 - "The Fawn Hooded (FH) rat strain possesses a genetic platelet storage pool deficiency which leads to an impaired capacity for platelets to store and release serotonin. " 07/01/1988 - "The mutation causes increased bleeding time accompanied by symptoms of platelet storage pool deficiency (SPD), including decreased platelet serotonin and decreased visibility of dense granules as analysed by electron microscopy of unfixed platelets. "
2.	Adenosine Diphosphate (ADP)IBA 03/01/1991 - "Although the mean intraplatelet ADP level improved during treatment, it always remained below the normal range documenting persistence of the delta-storage pool deficiency. " 11/01/2012 - "The platelet count of patients with congenital delta-storage pool deficiency, who lack secretable adenosine diphosphate, was significantly lower than that of patients with other platelet function disorders, confirming the important role of secretable adenosine diphosphate in platelet formation. " 05/15/1996 - "Correction of the platelet adhesion defect in delta-storage pool deficiency at elevated hematocrit--possible role of adenosine diphosphate." 06/01/1984 - "These patients had acquired storage pool deficiency of platelet dense granule ADP that correlated directly with platelet dysfunction, as measured by prolongation of bleeding time. " 11/01/1981 - "These findings suggest that in feline CH platelets, as in platelets from CH mink and cattle, there is storage pool deficiency that is virtually complete, and the virtual absence of ADP and 5HT may in part account for the abnormal hemostasis. "
3.	Adenine NucleotidesIBA 12/29/1984 - "A storage pool deficiency of adenine nucleotides was considered to be responsible for abnormal platelet function in patients with myeloproliferative disorders." 02/01/1999 - "Evidence that secreted dense granule adenine nucleotides mediate part of the agonist-induced cytosolic calcium ([Ca2+]i) responses in human platelets was obtained from comparisons of fura-2-loaded platelets from normal subjects and from patients with a form of platelet storage pool deficiency (SPD) in which the secretory dense granules and their contents are virtually absent. " 06/01/1991 - "The defect(s) in aggregation of platelets from beige mice was associated with a dense granule storage pool deficiency and decreased stores of serotonin and adenine nucleotides in platelets. " 02/01/1986 - "The storage pool deficiency of adenine nucleotides in this disease found by others on basis of release experiments (3) can thus be diagnosed by rapid and simple measurements of Ap4A in whole blood using the advantage of Ap4A being a specific components stored in dense granules." 01/01/1998 - "For example, platelet storage pool deficiency (SPD) is due to a marked decrease or absence of the organelles storing adenine nucleotides, serotonin, and calcium destined for secretion during the platelet release reaction. "
4.	CalciumIBA 02/01/1999 - "Evidence that secreted dense granule adenine nucleotides mediate part of the agonist-induced cytosolic calcium ([Ca2+]i) responses in human platelets was obtained from comparisons of fura-2-loaded platelets from normal subjects and from patients with a form of platelet storage pool deficiency (SPD) in which the secretory dense granules and their contents are virtually absent. " 01/01/1998 - "For example, platelet storage pool deficiency (SPD) is due to a marked decrease or absence of the organelles storing adenine nucleotides, serotonin, and calcium destined for secretion during the platelet release reaction. " 03/01/1997 - "Platelet prothrombinase activity and intracellular calcium responses in patients with storage pool deficiency, glycoprotein IIb-IIIa deficiency, or impaired platelet coagulant activity--a comparison with Scott syndrome."
5.	Erythropoietin FDA Link 05/15/1998 - "Correction of uremic platelet serotonin (5-HT) storage pool deficiency is one of the very early hemostatic effects of erythropoietin (Epo) therapy. " 01/01/1998 - "Recombinant human erythropoietin (EPO) not only ameliorates the anemia of renal failure but also modulates platelet function and corrects uremic platelet serotonin (5-hydroxytryptamine, 5-HT) storage pool deficiency. "
6.	Proteins (Proteins, Gene)IBA 07/01/1999 - "Abnormal expression and subcellular distribution of subunit proteins of the AP-3 adaptor complex lead to platelet storage pool deficiency in the pearl mouse." 06/01/1991 - "Results indicate that the biochemical defect(s) in platelet function in beige mice is partially attributable to storage pool deficiency and does not result in an absolute defect in phosphorylation of 40- and 20-kD proteins." 06/01/2001 - "These unique features of their processing led us to study these proteins in alpha delta storage pool deficiency (alphadelta-SPD) and grey platelet syndrome (GPS, alpha-SPD), two conditions known to impair alpha-granule protein storage. " 07/02/2004 - "The genes encoding these proteins are defective in inbred mouse strains that serve as models of Hermansky-Pudlak syndrome (HPS), a genetic disorder characterized by hypopigmentation and platelet storage pool deficiency. " 03/01/2010 - "We now demonstrate by immunoblotting and immunofluorescence microscopy that platelet MRP4 is absent in two patients with a platelet delta-storage pool deficiency (delta-SPD)-like phenotype with reduced platelet adenine nucleotide (AN) but normal serotonin levels, whereas their other membrane marker proteins of platelet granules were normally expressed and localized. "
7.	Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric 11/01/1999 - "Measurements were repeated I and 4 hours after intravenous infusion of desmopressin in six delta-storage pool deficiency and eight primary secretion defect patients. " 09/10/2002 - "The bleeding tendency that is associated with this autosomal recessive disease is caused by storage-pool deficiency and has been reported to be controllable by prophylactic administration of 1-desamino 8D-arginine (desmopressin, DDAVP). " 01/01/1988 - "1-Desamino-8-D-arginine vasopressin (desmopressin) shortens the bleeding time in storage pool deficiency." 10/01/1995 - "To examine the impact of the underlying defective platelet mechanism on the response to 1-desamino-8-D-arginine vasopressin (DDAVP, Desmopressin), we studied the effect of intravenous infusion of 0.3 microgram/kg of DDAVP in a randomized double blind placebo-controlled trial with cross-over in 18 carefully characterized patients with congenital platelet defects (CPD) and BT > or = 9 min. Eleven patients had normal dense granule stores and normal thromboxane A2 (TxA2) production (Group I), 3 patients had normal granule stores but impaired TxA2 production (Group II), and 4 had delta-storage pool deficiency (Group III). "
8.	Vasopressins (Vasopressin)IBA 01/01/1988 - "1-Desamino-8-D-arginine vasopressin (desmopressin) shortens the bleeding time in storage pool deficiency." 10/01/1995 - "To examine the impact of the underlying defective platelet mechanism on the response to 1-desamino-8-D-arginine vasopressin (DDAVP, Desmopressin), we studied the effect of intravenous infusion of 0.3 microgram/kg of DDAVP in a randomized double blind placebo-controlled trial with cross-over in 18 carefully characterized patients with congenital platelet defects (CPD) and BT > or = 9 min. Eleven patients had normal dense granule stores and normal thromboxane A2 (TxA2) production (Group I), 3 patients had normal granule stores but impaired TxA2 production (Group II), and 4 had delta-storage pool deficiency (Group III). "
9.	Thromboxane A2 (A2, Thromboxane)IBA 08/01/1998 - "A small proportion of these patients have deficiency of granule stores (storage pool deficiency [SPD]) or impaired production of thromboxane A2; in most, the mechanisms underlying the platelet dysfunction are unknown. " 10/01/1995 - "To examine the impact of the underlying defective platelet mechanism on the response to 1-desamino-8-D-arginine vasopressin (DDAVP, Desmopressin), we studied the effect of intravenous infusion of 0.3 microgram/kg of DDAVP in a randomized double blind placebo-controlled trial with cross-over in 18 carefully characterized patients with congenital platelet defects (CPD) and BT > or = 9 min. Eleven patients had normal dense granule stores and normal thromboxane A2 (TxA2) production (Group I), 3 patients had normal granule stores but impaired TxA2 production (Group II), and 4 had delta-storage pool deficiency (Group III). "
10.	Quinacrine (Mepacrine)IBA 05/01/2000 - "Platelet aggregation profile, mepacrine fluorescence test, flow cytometry and transmission electron microscopy studies led to the diagnosis of delta-storage pool deficiency (SPD) A few months later, she developed hyperleucocytosis with immature granulocytes and erythroblasts. " 05/01/1987 - "Mepacrine uptake into platelets and bone marrow megakaryocytes was analyzed to further characterize the dense granule defects in a group of seven mouse pigment mutants that have characteristics of platelet storage pool disease (SPD). "

Therapies and Procedures

1.	Intravenous Infusions 11/01/1999 - "Measurements were repeated I and 4 hours after intravenous infusion of desmopressin in six delta-storage pool deficiency and eight primary secretion defect patients. " 10/01/1995 - "To examine the impact of the underlying defective platelet mechanism on the response to 1-desamino-8-D-arginine vasopressin (DDAVP, Desmopressin), we studied the effect of intravenous infusion of 0.3 microgram/kg of DDAVP in a randomized double blind placebo-controlled trial with cross-over in 18 carefully characterized patients with congenital platelet defects (CPD) and BT > or = 9 min. Eleven patients had normal dense granule stores and normal thromboxane A2 (TxA2) production (Group I), 3 patients had normal granule stores but impaired TxA2 production (Group II), and 4 had delta-storage pool deficiency (Group III). "
2.	Cardiopulmonary Bypass (Heart-Lung Bypass) 04/01/1978 - "In vitro aggregation tests with protamine sulfate and hemoglobin solutions, as well as the 14C-serotonin studies and electron micrographs, suggest that platelets acquire storage pool deficiency and an abnormal membrane during cardiopulmonary bypass."
3.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 05/01/1986 - "Progenitor cell defect correctable by bone marrow transplantation in five independent mouse models of platelet storage pool deficiency."