Bleeding problems are symptomatic of platelet delta-storage pool diseases (SPDs) such as Hermansky-Pudlak syndrome. Although at present no cure is available for delta-SPD, early diagnosis is of great importance for prophylactic and supportive treatment. This study tested the usefulness of a flow cytometric assay for platelet serotonin in children. The assay was used to diagnose delta-SPD in a 10-year-old girl. Platelet serotonin levels were significantly lower in the patient than in all healthy control subjects (10 children and 10 adults). The serotonin results were supported by traditional tests, which are transmission electron microscopy of whole mounts and adenosine triphosphate release by lumi-aggregometry. The flow cytometric serotonin assay is a major improvement to current pediatric diagnostics. The advantages of this test are small sample volume of fresh or fixed/frozen platelets, availability of objective results within 2 hours of obtaining the blood sample, and automated analysis by flow cytometry.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1309/3KRYCPNAPDTVFWGY | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Challenges of platelet electron microscopy in the diagnosis of platelet delta storage pool disorder.
Pathology
October 2024
Department of Haematology, Pathology Queensland, Brisbane, Qld, Australia.
Mepacrine Flow Cytometry Assay for the Diagnosis of Platelet δ-granule Defects: Literature Review on Methods-Towards a Shared Detailed Protocol.
Thromb Haemost
October 2024
Department of Laboratory Medicine, Hematology Laboratory, CHU UCL Namur, Yvoir, Belgium.
Accurate assessment of platelet secretion is essential for the diagnosis of inherited or acquired platelet function disorders and more specifically in identifying δ-storage pool disease. Mepacrine, a fluorescent dye, specifically accumulates in platelet δ-granules. The mepacrine flow cytometry (mepacrine FCM) assay has been used for more than half a century in the clinical laboratory as a diagnostic tool for platelet δ-granule disorders.
View Article and Find Full Text PDFDelta Storage Pool Deficiency: A Pediatric Case Report and Review of the Literature.
Cureus
December 2023
Pediatric Oncology, Fundación Santa Fe de Bogotá, Bogotá, COL.
Platelet storage deficiencies are a heterogeneous group of bleeding disorders of variable severity caused by decreased number or content of platelet granules. We present the case of a 10-year-old patient with no personal history of previous bleeding who was admitted to the emergency department due to menorrhagia and mucocutaneous pallor. Common disorders of primary and secondary hemostasis were ruled out.
View Article and Find Full Text PDFMulticolor flow cytometry in clinical samples for platelet signaling assessment.
Res Pract Thromb Haemost
May 2023
CHU de Toulouse, Laboratoire d'Hématologie, Toulouse, France.
Background: Availability of multichannel cytometers and specific commercial antibodies makes flow cytometry a new option to simultaneously assess multiple intracellular platelet signaling pathways for clinical purposes, in small volume of blood or low platelet count.
Objectives: To describe a multicolor flow cytometry with fluorescent barcoding technique for screening signaling pathways downstream membrane receptors of major platelet agonists (adenosine diphosphate, thrombin, thromboxane, and collagen).
Methods: By comparison with immunoblotting, we first selected the target phosphoproteins, AKT, P38MAPK, LIMK, and SPL76; the times of stimulation; and phosphoflow barcoding conditions.
Platelet Delta (δ)-Storage Pool Deficiency: A Case Series and Review of the Literature.
Hematol Rep
June 2023
Department of Hematology/Oncology, Indiana University School of Medicine, Muncie, IN 47303, USA.
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.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!