Platelet Delta granules storage pool deficiency in female patient with severe hemorrhagic syndrome
https://doi.org/10.35754/0234-5730-2023-68-1-90-97
Abstract
Introduction. Platelet dense granule disorders are a group of rare heterogeneous disorders of the blood coagulation system in which bleeding occurs due to functional and morphological disorders of platelet organelles accumulating phosphates and bioactive amines.
Aim — to present a clinical case of a 37-year-old patient with severe hemorrhagic syndrome.
Basic information. An observation of the occurrence of hemorrhagic manifestations of unspecified genesis in a patient is described. The results of 25 healthy volunteer examinations of both sexes were used as a control for testing methods of diagnosis of Platelet dense granule disorder. Methods of assessing the hemostasis system, platelet morphological features using electron microscopy, as well as platelet accumulation of mepacrine using a flow cytometer were studied. Platelet dense granule disorder was detected by electron microscopy and confirmed by flow cytometry in a patient with severe hemorrhagic manifestations, in whom the diagnosis was not verified for a prolonged period of time.
Keywords
thrombocytopathy,
platelet hemostasis,
dense granules,
platelet δ-granules storage pool deficiency,
electron microscopy of platelets
Supporting agencies: The study had no sponsorship
About the Authors
I. V. Kosheed
Altai Regional Hospital
Russian Federation
Russian Federation
Irina V. Kosheed, Clinical Diagnostitian
Laboratory of Hemostasis Pathology
656024
Barnaul
A. N. Mamaev
Altai Regional Hospital; Altai Branch of National Medical Research Center for Hematology
Russian Federation
Russian Federation
Andrey N. Mamaev, Dr. Sci. (Med.), Senior Researcher
656024
Barnaul
Y. G. Motin
Altai Regional Hospital
Russian Federation
Russian Federation
Yuri G. Motin, Dr. Sci. (Med.), Professor, Pathologist
656024
Barnaul
A. V. Kudinov
Altai Branch of National Medical Research Center for Hematology
Russian Federation
Russian Federation
Alexey V. Kudinov, Cand. Sci. (Biol.), Senior Researcher
656024
Barnaul
A. P. Momot
Altai Branch of National Medical Research Center for Hematology
Russian Federation
Russian Federation
Andrey P. Momot, Dr. Sci. (Med.), Professor, Director
656024
Barnaul
T. N. Babaeva
Novosibirsk State Medical University
Russian Federation
Russian Federation
Tatiana N. Babaeva, Cand. Sci. (Med.), Teaching Assistant
Department of Therapy, Hematology and Transfusiology
630031
Novosibirsk
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For citations:
Kosheed I.V., Mamaev A.N., Motin Y.G., Kudinov A.V., Momot A.P., Babaeva T.N. Platelet Delta granules storage pool deficiency in female patient with severe hemorrhagic syndrome. Russian journal of hematology and transfusiology. 2023;68(1):90-97. (In Russ.) https://doi.org/10.35754/0234-5730-2023-68-1-90-97
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