Platelet storage pool deficiency

File:Iron deficiency anemia blood film.jpg

The symptoms individuals with platelet storage pool deficiency may experience include the following:

• Easy bruising
• Nose bleeds
• Bleeding from gums
• Heavy or prolonged menstrual bleeding (menorrhagia) or bleeding after childbirth
• Abnormal bleeding after surgery, circumcision, or dental work

Severity can vary widely from person to person, and individuals with platelet storage pool deficiency may not experience all of the above symptoms.{{clear|left}}

File:Hairy cell leukemia.jpg

Platelet storage pool deficiency can be acquired or inherited. Inheritance may be autosomal dominant or autosomal recessive, depending on the specific disorder.

Some of the causes of platelet storage pool deficiency when acquired are:{{Cite journal |last=Konkle |first=Barbara |date=10 December 2011 |title=Acquired disorders of platelet function |url=https://ashpublications.org/hematology/article/2011/1/391/96432/Acquired-Disorders-of-Platelet-Function |journal=Hematology Am Soc Hematol Educ Program |volume=2011 |issue=1 |pages=391–396 |doi=10.1182/asheducation-2011.1.391 |pmid=22160063 |via=Ash Publications|url-access=subscription }}

::::::::*Hairy-cell leukemia

::::::::*Cardiovascular bypass

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File:Platelet structure.png

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{{citation needed|date=November 2017}} 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.{{cite journal|last1=Blair|first1=Price|last2=Flaumenhaft|first2=Robert|title=Platelet alpha-granules: basic biology and clinical correlates|journal=Blood Reviews|date=2009|volume=23|issue=4|pages=177–189|doi=10.1016/j.blre.2009.04.001|issn=1532-1681|pmid=19450911|pmc=2720568}}

File:First and second wave of platelet aggregation.png-initiated aggregation. The second wave is absent in platelet storage pool deficiency.]]

The diagnosis of this condition can be done via the following:{{Cite web |title=Alpha Delta Granule Deficiency |url=https://rarediseases.info.nih.gov/diseases/5034/disease |access-date=2017-10-28 |website=rarediseases.info.nih.gov |language=en}}

• Flow cytometry
• Bleeding time analysis
• Platelet aggregation function study:

{{Comprehensive table of platelet aggregation disorders and agonists}}

This condition may involve the alpha granules or the dense granules.{{cite book|author1=William B. Coleman|author2=Gregory J. Tsongalis|title=Molecular pathology: the molecular basis of human disease|url=https://books.google.com/books?id=btloapOq85wC&pg=PA258|access-date=2 November 2010|year=2009|publisher=Academic Press|isbn=978-0-12-374419-7|pages=258–}} Some common inherited disorders associated with each include the following:

File:FACS-toestel.JPG

• Platelet alpha-granules
• Gray platelet syndrome{{Cite web |last= |first= |title=Gray platelet syndrome |url=http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=721 |access-date=2017-10-29 |website=Orphanet |language=en}}
• Quebec platelet disorder{{cite web|title=OMIM Entry - # 601709 - Quebec Platelet Disorder |url=https://www.omim.org/entry/601709|website=www.omim.org|access-date=29 October 2017|language=en-us}}
• Dense granules
• δ-Storage pool deficiencyKaushansky 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}}
• Hermansky–Pudlak syndrome{{Cite book|chapter-url=https://www.ncbi.nlm.nih.gov/books/NBK1287/|title=GeneReviews|last1=Huizing|first1=Marjan|last2=Malicdan|first2=May Christine V.|last3=Gochuico|first3=Bernadette R.|last4=Gahl|first4=William A.|date=1993|publisher=University of Washington, Seattle|editor-last=Adam|editor-first=Margaret P.|location=Seattle (WA)|pmid=20301464|editor-last2=Ardinger|editor-first2=Holly H.|editor-last3=Pagon|editor-first3=Roberta A.|editor-last4=Wallace|editor-first4=Stephanie E.|editor-last5=Bean|editor-first5=Lora J.H.|editor-last6=Mefford|editor-first6=Heather C.|editor-last7=Stephens|editor-first7=Karen|editor-last8=Amemiya|editor-first8=Anne|editor-last9=Ledbetter|editor-first9=Nikki|chapter=Hermansky-Pudlak Syndrome}}update 2017
• Chédiak–Higashi syndrome{{cite web |last1= |first1= |title=Chédiak Higashi syndrome |url=http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=167 |access-date=29 October 2017 |website=Orphanet |language=en}}

Platelet storage pool deficiency usually requires no daily treatment, although many individuals with heavy menstrual bleeding take hormonal contraceptives to reduce menstrual symptoms.{{Cite web |last= |first= |title=More Than a Bloody Nose: Platelet Storage Pool Deficiency |url=https://www.chop.edu/news/more-bloody-nose-platelet-storage-pool-deficiency |access-date=2024-09-02 |website=Children's Hospital of Philadelphia |language=en}} However, management of uncontrolled bleeding consists of antifibrinolytic medications or transfusion of normal blood products. Additionally, caution should be taken with usage of NSAIDS, since they thin the blood and further impair clotting.{{cite journal|last1=Kirchmaier|first1=Carl Maximilian|last2=Pillitteri|first2=Daniele|title=Diagnosis and Management of Inherited Platelet Disorders|journal=Transfusion Medicine and Hemotherapy|date=October 2010|volume=37|issue=5|pages=237–246|doi=10.1159/000320257|issn=1660-3796|pmc=2980508|pmid=21113246}}

• Hypocoagulability
• Hypercoagulability

{{Reflist}}

• {{cite journal|last1=Sandrock|first1=Kirstin|last2=Zieger|first2=Barbara|title=Current Strategies in Diagnosis of Inherited Storage Pool Defects|journal=Transfusion Medicine and Hemotherapy|date=2010|volume=37|issue=5|pages=248–258|doi=10.1159/000320279|issn=1660-3796|pmc=2980509|pmid=21113247}}
• {{cite book|last1=Gresele|first1=Paolo|last2=Fuster|first2=Valentin|last3=Lopez|first3=Jose A.|last4=Page|first4=Clive P.|last5=Vermylen|first5=Jos|title=Platelets in Hematologic and Cardiovascular Disorders: A Clinical Handbook|date=2007|publisher=Cambridge University Press|isbn=9781139468763|url=https://books.google.com/books?id=kB4C84kCDZEC&q=Platelet+storage+pool+deficiency&pg=PA220|access-date=26 November 2017|language=en}}

• [https://www.ncbi.nlm.nih.gov/pubmed/ PubMed]

{{Medical resources

| DiseasesDB =

| ICD10 = {{ICD10|D|69|1|d|50}}

| ICD9 =

| ICDO =

| OMIM = 185050

| MedlinePlus =

| eMedicineSubj =

| eMedicineTopic =

| MeshID = D010981

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{{Diseases of megakaryocytes|us=y}}

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Category:Coagulopathies