Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children66010.24287/1726-1708-2022-21-3-142-146LITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫThe electron microscopy contribution to platelet structural pathology investigationВклад электронной микроскопии в исследование нарушений морфологии тромбоцитовhttps://orcid.org/0000-0002-2930-8768ObydennyiS. I.ОбыденныйС. И.
Russian Federation

Sergey I. Obydennyi, a researcher at the Laboratory of Cell Hemostasis and Thrombosis

1 Samory Mashela St., Moscow 117997, Russia

Обыденный Сергей Иванович, научный сотрудник лаборатории клеточного гемостаза и тромбоза

117997, Москва, ул. Саморы Машела, 1

sergey.obidenniy@fccho-moscow.ruhttps://orcid.org/0000-0001-9252-6808KireevI. I.КиреевИ. И.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-8128-7757PanteleevM. A.ПантелеевМ. А.
Russian Federation

Moscow

Москва

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of the Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииCenter for Theoretical Problems of Physicochemical Pharmacology, RASФГБУН «Центр теоретических проблем физико-химической фармакологии» РАНLomonosov Moscow State UniversityФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова»151020222131421461510202215102022Copyright ©; 2022, «D. Rogachev NMRCPHOI»Copyright ©; 2022, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2022«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/660

This article discusses the role of electron microscopy in the diagnosis and study of morphological changes that cause platelet structural abnormalities in a variety of congenital diseases. Morphological abnormalities can be divided into the abnormalities of the platelet cytoskeleton, of alpha and dense granules, and membrane abnormalities. Our paper describes ultrastructural platelet defects in Wiskott–Aldrich syndrome, MYH9-associated syndromes, gray platelet syndrome, Hermansky–Pudlak syndrome, Paris–Trousseau syndrome, Chediak–Higashi syndrome.

В данной статье рассматривается роль электронной микроскопии в диагностике и изучении морфологических изменений при врожденных заболеваниях, вызывающих нарушения строения тромбоцитов. Морфологические нарушения могут быть разделены на патологии цитоскелета тромбоцитов, альфа- и плотных гранул, а также изменения мембраны. В обзоре описываются ультраструктурные дефекты тромбоцитов при синдроме Вискотта–Олдрича, MYH9-ассоциированных синдромах, синдроме серых тромбоцитов, синдроме Германского–Пудлака, синдроме Пари–Труссо и синдроме Чедиака–Хигаши.

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