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Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature
Hereditary platelet delta (δ)-storage pool deficiency is a rare condition in which there are fewer dense granules in platelets disrupting primary hemostasis. It can cause a mild–moderate bleeding tendency with normal coagulation studies; hence, it is an underdiagnosed diagnostic challenge. The autho...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366910/ https://www.ncbi.nlm.nih.gov/pubmed/37489371 http://dx.doi.org/10.3390/hematolrep15030041 |
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author | Beirat, Amir F. Menakuru, Sasmith R. Kalra, Maitri |
author_facet | Beirat, Amir F. Menakuru, Sasmith R. Kalra, Maitri |
author_sort | Beirat, Amir F. |
collection | PubMed |
description | Hereditary platelet delta (δ)-storage pool deficiency is a rare condition in which there are fewer dense granules in platelets disrupting primary hemostasis. It can cause a mild–moderate bleeding tendency with normal coagulation studies; hence, it is an underdiagnosed diagnostic challenge. The authors present three patients with hereditary platelet delta (δ)-storage pool deficiency who had heavy menstrual bleeding, excessive bleeding following surgery, mucocutaneous bleeding, and a bleeding score greater than or equal to 6. These cases reveal the susceptibility of underdiagnosing platelet disorders and the significance of utilizing a bleeding assessment tool to help guide further workup with transmission electron microscopy to visualize the fewer dense granules in platelets. Although bleeding is typically moderate, it can be severe in certain scenarios, like after mucosal surgeries, and can lead to death, highlighting the importance of the condition’s recognition and prophylactic treatment. |
format | Online Article Text |
id | pubmed-10366910 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-103669102023-07-26 Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature Beirat, Amir F. Menakuru, Sasmith R. Kalra, Maitri Hematol Rep Case Report Hereditary platelet delta (δ)-storage pool deficiency is a rare condition in which there are fewer dense granules in platelets disrupting primary hemostasis. It can cause a mild–moderate bleeding tendency with normal coagulation studies; hence, it is an underdiagnosed diagnostic challenge. The authors present three patients with hereditary platelet delta (δ)-storage pool deficiency who had heavy menstrual bleeding, excessive bleeding following surgery, mucocutaneous bleeding, and a bleeding score greater than or equal to 6. These cases reveal the susceptibility of underdiagnosing platelet disorders and the significance of utilizing a bleeding assessment tool to help guide further workup with transmission electron microscopy to visualize the fewer dense granules in platelets. Although bleeding is typically moderate, it can be severe in certain scenarios, like after mucosal surgeries, and can lead to death, highlighting the importance of the condition’s recognition and prophylactic treatment. MDPI 2023-06-29 /pmc/articles/PMC10366910/ /pubmed/37489371 http://dx.doi.org/10.3390/hematolrep15030041 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Beirat, Amir F. Menakuru, Sasmith R. Kalra, Maitri Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title | Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title_full | Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title_fullStr | Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title_full_unstemmed | Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title_short | Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature |
title_sort | platelet delta (δ)-storage pool deficiency: a case series and review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10366910/ https://www.ncbi.nlm.nih.gov/pubmed/37489371 http://dx.doi.org/10.3390/hematolrep15030041 |
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