Гематологические проявления болезни Гоше (обзор литературы)

Болезнь Гоше - мультисистемное заболевание, развивающееся вследствие дефицита глюкоцереброзидазы и накопления глюкозилцерамида и глюкозилсфингозина в лизосомах фагоцитирующих макрофагов. Представлена характеристика всех типов болезни Гоше. Подробное внимание в обзоре уделяется анализу гематологических проявлений болезни Гоше, приводятся результаты современных исследований, объясняющие их появление при данном заболевании.

тромбоцитопения, геморрагический диатез, анемия, спленомегалия, гаммапатии и злокачественные новообразования, болезнь Гоше, thrombocytopenia, haemorrhagic diathesis, anaemia, splenomegaly, gammopathies and malignancies, Gaucheґs disease

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