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Gray Platelet Syndrome

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A rare, inherited platelet disorder characterized by a selective deficiency in the number and contents of platelet alpha-granules. It is associated with THROMBOCYTOPENIA, enlarged platelets, and prolonged bleeding time.

Also Known As:

Grey Platelet Syndrome; Platelet alpha-Granule Deficiency; Gray Platelet Syndromes; Grey Platelet Syndromes; Platelet Syndromes, Grey; Platelet alpha Granule Deficiency; Platelet alpha-Granule Deficiencies; Syndrome, Gray Platelet; Syndrome, Grey Platelet; Syndromes, Gray Platelet; alpha-Granule Deficiencies, Platelet; alpha-Granule Deficiency, Platelet

Networked: 69 relevant articles (2 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Platelet Storage Pool Deficiency
2.	Hemorrhage
3.	Thrombasthenia (Glanzmann Thrombasthenia)
4.	Thrombocytopenia (Thrombopenia)
5.	Hermanski-Pudlak Syndrome (Hermansky-Pudlak Syndrome)

Experts

1.	Zieger, Barbara: 2 articles (10/2015 - 01/2014)
2.	Nurden, P: 2 articles (06/2015 - 07/2011)
3.	White, James G: 2 articles (01/2013 - 09/2004)
4.	Pecci, A: 2 articles (08/2012 - 03/2007)
5.	Balduini, C L: 2 articles (08/2012 - 03/2007)
6.	Nurden, Alan T: 2 articles (07/2009 - 07/2004)
7.	Nurden, Paquita: 2 articles (07/2009 - 07/2004)
8.	Rensing-Ehl, Anne: 1 article (10/2015)
9.	Schwarz, Klaus: 1 article (10/2015)
10.	Speckmann, Carsten: 1 article (10/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Gray Platelet Syndrome:

1.	Proteins (Proteins, Gene)IBA 08/01/1992 - "Gray platelet syndrome is a rare (about 40 cases published), inherited disorder characterized by a marked decrease or absence of platelet alpha-granules and platelet specific alpha-granule proteins. " 04/01/1985 - "The grey platelet syndrome is a rare inherited disorder characterized by a marked decrease or absence of alpha-granules and of platelet-specific alpha-granule proteins. " 02/01/2011 - "Several patients have been described with platelet PF4 deficiency, including the gray platelet syndrome, characterized by a deficiency of α-granule proteins. " 01/01/1992 - "Platelets from a patient having a gray platelet syndrome, characterized by a deficiency in platelet alpha-granules and in the alpha-granule secretory proteins, did not show any deficiency of BSP, suggesting that immunoreactive BSP present in platelets is not endogenously synthesized by megakaryocytes but rather originates from plasma by endocytosis." 10/01/1991 - "This observation allows to conclude that alpha-granule membrane is formed in the gray platelet syndrome, but that there is a storage defect of alpha-granule soluble proteins, possibly due to an abnormal targetting of these proteins to the alpha-granule. "
2.	Thrombin FDA Link 04/01/1987 - "Grey platelet syndrome: studies on platelet alpha-granules, lysosomes and defective response to thrombin." 10/01/1987 - "Washed platelets isolated from one patient suffering from the inherited grey platelet syndrome were studied during thrombin-induced activation. " 02/01/1996 - "Binding sites of 125I-labelled LYP7, which was greatly increased on thrombin-stimulated (2 U/ml) washed platelets (10825 +/- 2886, mean +/- SD) Kd = 1.5 +/- 0.5 nM) compared to resting platelets (2801 +/- 1278, mean +/- SD) (Kd = 1.5 +/- 0.6 nM), was found to be normal on thrombin-stimulated platelets taken from a patient with grey platelet syndrome or a patient with Glanzmann thrombasthenia. " 03/01/2007 - "Defective platelet responsiveness to thrombin and protease-activated receptors agonists in a novel case of gray platelet syndrome: correlation between the platelet defect and the alpha-granule content in the patient and four relatives." 08/04/1987 - "Thrombin-induced platelet factor Va formation in patients with a gray platelet syndrome."
3.	May-Hegglin anomalyIBA 01/01/1995 - "Abnormal organization of platelet microtubules is associated with abnormal platelet formation in hereditary macrothrombocytopenias such as the gray platelet syndrome, May-Hegglin anomaly, and Epstein's syndrome, and that of the Wistar Furth rat, suggesting that aberrant microtubule organization may contribute to defective platelet formation in these clinical entities. " 10/01/1994 - "This review summarizes major clinical and laboratory features of three GPDs (Bernard-Soulier syndrome, May-Hegglin anomaly and gray platelet syndrome). " 01/01/2003 - "Bernard-Soulier syndrome, grey platelet syndrome and May-Hegglin anomaly are the most common giant platelet thrombocytopenias. " 01/01/2014 - "Application with patient blood reveals distinct abnormalities in thrombus formation in patients with severe combined immune deficiency, Glanzmann's thrombasthenia, Hermansky-Pudlak syndrome, May-Hegglin anomaly or grey platelet syndrome. " 10/01/1999 - "This priming effect was observed in patients with von Willebrand's disease, hemophilia A, May-Hegglin anomaly, gray platelet syndrome and Ehlers-Danlos syndrome. "
4.	Factor V (Coagulation Factor V)IBA 08/04/1987 - "The present study was initiated to establish the functional factor V concentration in platelets of patients with a mild bleeding disorder ascribed to a gray platelet syndrome. " 03/01/1997 - "In one of these patients this may be caused by an abnormality in platelet alpha-granule factor V distinct from that described for factor V Quebec, alpha delta-SPD, or alpha-SPD (gray platelet syndrome). " 06/01/2001 - "The storage defects in grey platelet syndrome and alphadelta-storage pool deficiency affect alpha-granule factor V and multimerin storage without altering their proteolytic processing."
5.	TubulinIBA 09/01/1984 - "In the present study platelets from patients with the Gray platelet syndrome (GPS), May-Hegglin anomaly (MHA), and Epstein's syndrome (ES) were examined in thin sections and negatively stained whole mounts, and by indirect immunofluorescence with a monoclonal antibody to tubulin for determination of the organization of their microtubule systems. "
6.	Peroxidase (Myeloperoxidase)IBA 02/01/1972 - "The present study has employed ultrastructural observations on normal platelets, cells from a child with the gray platelet syndrome and cytochemical localization of peroxidase in platelets to define the origin of the DTS and point out a specialized membrane complex in platelets formed by interaction of channels from the OCS and DTS. "
7.	P-SelectinIBA 07/01/1996 - "Deficiency of P-selectin in a patient with grey platelet syndrome." 12/01/1992 - "It was shown by immunoblotting that platelets from the patient with gray platelet syndrome contained normal amount of GP IIa, but strongly decreased amount of GMP-140 (14.5% of control)--membrane GP of platelet--granules." 03/01/1991 - "These findings in the severe alpha delta-SPD patient are in clear contrast to the observations of normal GMP-140 content in the three other alpha delta-SPD patients, and in patients with the gray platelet syndrome, reported previously by others. " 03/01/1997 - "Platelet alpha granule deficiency associated with decreased P-selectin and selective impairment of thrombin-induced activation in a new patient with gray platelet syndrome (alpha-storage pool deficiency)."
8.	Glycoproteins (Glycoprotein)IBA 07/01/2004 - "Severe deficiency of glycoprotein VI in a patient with gray platelet syndrome." 04/01/1982 - "Specific protein and glycoprotein deficiencies in platelets isolated from two patients with the gray platelet syndrome." 03/15/1990 - "Using an immunogold staining technique and electron microscopy, we investigated the localization of the alpha-granule pool of glycoprotein (GP) IIb-IIIa in normal platelets and maturing megakaryocytes (MK), in pathologic platelets from a patient with type I Glanzmann's thrombasthenia (GT), and from three patients with the gray platelet syndrome (GPS). " 06/01/1986 - "Platelets from patients with afibrinogenemia, Gray platelet syndrome, and the Cam Variant of thrombasthenia, a form of thrombasthenia with near normal levels of glycoprotein IIb/IIIa (GPIIb/IIIa), showed minimal augmentation of plasminogen binding to thrombin-stimulated platelets but normal binding to unstimulated platelets. "
9.	AntibodiesIBA 02/01/1994 - "The expression of these two Ca2+ATPases was compared by Western blotting using specific antibodies, which again emerged in similar amounts in normal and grey platelet syndrome platelets. " 12/01/1992 - "In patient with gray platelet syndrome binding of both antibodies was not decreased but even increased. " 10/01/1988 - "This 'pseudo grey platelet syndrome' was associated with platelet dense body and alpha granule secretion with no aggregation and occurred on removal of extracellular Ca2+. We discovered that a plasma factor was responsible which could be an immunoglobulin but which is clearly different from the EDTA-sensitive antibodies which cause platelet aggregation and agglutination. " 05/01/1988 - "Because thrombin aggregates afibrinogenemic platelets and platelets from patients with the gray platelet syndrome and because antibodies to fibrinogen inhibit thrombin-induced aggregation only at low concentrations of thrombin, the role of fibrinogen in the formation of thrombin-induced aggregates was investigated further with human platelets washed and resuspended in Tyrode-albumin solution containing apyrase, either with or without added Ca2+ (2 mmol/L). "
10.	von Willebrand FactorIBA 07/01/2009 - "Platelets from two patients with gray platelet syndrome, congenitally deficient in alpha-granules, showed sparse labeling of von Willebrand factor and fibrinogen confined to vestigial alpha-granules; however, localization of the TIMPs was unchanged. " 12/01/1985 - "Gray platelet syndrome: immunoelectron microscopic localization of fibrinogen and von Willebrand factor in platelets and megakaryocytes." 12/01/1985 - "An immunogold method was used for investigating the subcellular localization of von Willebrand factor (vWF) and fibrinogen (Fg) in platelets and cultured megakaryocytes from normal subjects and from three patients with the gray platelet syndrome (GPS), a rare congenital disorder characterized by the absence of alpha-granules. " 12/18/1987 - "Shortening of bleeding time by 1-deamino-8-arginine vasopressin (DDAVP) in the absence of platelet von Willebrand factor in Gray platelet syndrome."

Therapies and Procedures

1.	Hematopoietic Stem Cell Transplantation 03/01/2013 - "Successful hematopoietic engraftment with gray platelets after allogeneic hematopoietic stem cell transplantation from gray platelet syndrome donor."