Platelet δ-Storage Pool Disease: An Update
نویسندگان
چکیده
منابع مشابه
Diagnosing Platelet δ-Storage Pool Disease in Children by Flow Cytometry
Bleeding problems are symptomatic of platelet δ-storage pool diseases (SPDs) such as HermanskyPudlak syndrome. Although at present no cure is available for δ-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 δ-SPD in a 10-year-old gi...
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We report a new bleeding disease--storage pool deficiency (SPD) of platelets--in pigs from the Mayo swine colony of homozygous von Willebrand's disease (vWD) and of heterozygous carriers of vWD. Levels of factor VIII, von Willebrand factor antigen (vWF:Ag), and ristocetin cofactor (RCof) were similar in the vWD carriers and SPD pigs. The latter pigs, however, had bleeding times of 15 minutes or...
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Jacobsen syndrome and Paris-Trousseau Syndrome share similar congenital anomalies, thrombocytopenia, giant platelet alpha granules resulting from fusion of smaller organelles, and an 11q terminal deletion at 11q23.3. Similarities in the two cohorts have suggested that the Paris-Trousseau Syndrome is a variant of Jacobsen syndrome, or the same disorder. The present study has pointed out a signif...
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The antiestrogenic drug tamoxifen, used in patients with breast cancer, is associated with an increase in arterial and venous thrombotic events, the mechanism of which is not clearly understood. We report a case of a lady who presented with new bruising and prolonged bleeding following a tooth extraction 4-6 weeks after starting tamoxifen. Investigations were consistent with an acquired platele...
متن کاملImmature dense granules in platelets from mice with platelet storage pool disease.
Mepacrine uptake into platelets and bone marrow megakaryocytes was analyzed to further characterize the dense granule defects in a group of seven mouse pigment mutants that have characteristics of platelet storage pool disease (SPD). In contrast to our previous studies using electron microscopy, this method revealed that all mutants had normal numbers of dense granules. However, total mepacrine...
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ژورنال
عنوان ژورنال: Journal of Clinical Medicine
سال: 2020
ISSN: 2077-0383
DOI: 10.3390/jcm9082508