Platelet storage pool deficiency
| Platelet storage pool deficiency | |
|---|---|
| Specialty | Hematology  |
Platelet storage pool deficiency is a type of coagulopathy characterized by defects in the granules in platelets, particularly a lack of granular non-metabolic ADP.[1] Patients with ADP deficient "Storage Pool Disease" present a prolonged bleeding time due to impaired aggregation response to fibrillar collagen.
It may involve the alpha granules or the dense granules.[2]
Conditions
Examples include:
- Platelet alpha-granules
- Dense granules
- δ-Storage pool deficiency[4]
- Hermansky-Pudlak syndrome[5]
- Chediak-Higashi syndrome[6]
References
- ^ Alan D. Michelson (2007). Platelets. Burlington, MA: Academic Press/Elsevier. p. 313. ISBN 0-12-369367-5.
- ^ William B. Coleman; Gregory J. Tsongalis (2009). Molecular pathology: the molecular basis of human disease. Academic Press. pp. 258–. ISBN 978-0-12-374419-7. Retrieved 2 November 2010.
- ^ Hayward CP, Weiss HJ, Lages B, et al. (June 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". Br. J. Haematol. 113 (4): 871–7. doi:10.1046/j.1365-2141.2001.02833.x. PMID 11442477.
- ^ Kaushansky K, Lichtman M, Beutler E, Kipps T, Prchal J, Seligsohn U. (2010; edition 8: pages 1946–1948) Williams Hematology. McGraw-Hill. ISBN 978-0-07-162151-9
- ^ Huizing M, Anikster Y, Fitzpatrick DL, et al. (November 2001). "Hermansky-Pudlak syndrome type 3 in Ashkenazi Jews and other non-Puerto Rican patients with hypopigmentation and platelet storage-pool deficiency". Am. J. Hum. Genet. 69 (5): 1022–32. doi:10.1086/324168. PMC 1274349. PMID 11590544.
- ^ Novak EK, McGarry MP, Swank RT (November 1985). "Correction of symptoms of platelet storage pool deficiency in animal models for Chediak-Higashi syndrome and Hermansky-Pudlak syndrome". Blood. 66 (5): 1196–201. PMID 3902123.
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