Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature.

IF 1.1 Q4 HEMATOLOGY Hematology Reports Pub Date : 2023-06-29 DOI:10.3390/hematolrep15030041
Amir F Beirat, Sasmith R Menakuru, Maitri Kalra
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引用次数: 0

Abstract

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.

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血小板δ (δ)-储存池缺陷:一个案例系列和文献综述。
遗传性血小板δ (δ)储存池缺乏症是一种罕见的情况,其中血小板中致密颗粒较少,干扰原发性止血。它可以引起轻度至中度出血倾向与正常凝血研究;因此,这是一个诊断不足的挑战。作者报告了3例遗传性血小板δ (δ)储存池缺乏症患者,这些患者有大量月经出血,术后出血过多,皮肤粘膜出血,出血评分大于或等于6。这些病例揭示了血小板疾病诊断不足的易感性,以及利用出血评估工具帮助指导进一步检查的意义,通过透射电子显微镜观察血小板中较少的致密颗粒。虽然出血通常是中度的,但在某些情况下,如粘膜手术后,出血可能会很严重,并可能导致死亡,这凸显了识别和预防性治疗这种疾病的重要性。
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来源期刊
Hematology Reports
Hematology Reports HEMATOLOGY-
CiteScore
0.90
自引率
0.00%
发文量
47
审稿时长
10 weeks
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