SYNOPSIS IS A RARE CASE OF ORPHAN CO-MORBIDITY. EXPERIENCE IN TREATING A PATIENT WITH APLASTIC AND CLONOXYSMAL NOCTURNAL HEMOGLOBINURIA

Paroxysmal nocturnal hemoglobinuria (APG) is a rare clonal disease, an acquired form of hemolytic anemia from the group of rare (orphan) diseases. A characteristic clinical manifestation with a significant APG clone (usually more than 10 % of the total number of blood cells) is chronic intravascular...

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Published inМедицина в Кузбассе Vol. 23; no. 2; pp. 72 - 75
Main Authors Дмитрий Михайлович Неверов, Марина Владимировна Косинова, Светлана Ивановна Елгина, Елена Владимировна Рудаева, Кира Борисовна Мозес, Наталья Степановна Черных
Format Journal Article
LanguageRussian
Published The Publishing House Medicine and Enlightenment 01.06.2024
Subjects
апластическая анемия
лечение
пароксизмальная ночная гемоглобинурия
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ISSN1819-0901
2588-0411

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Summary:Paroxysmal nocturnal hemoglobinuria (APG) is a rare clonal disease, an acquired form of hemolytic anemia from the group of rare (orphan) diseases. A characteristic clinical manifestation with a significant APG clone (usually more than 10 % of the total number of blood cells) is chronic intravascular hemolysis, which, with its high activity, leads to the development of anemia, an increased tendency to thrombosis and a number of organ disorders. Manifestations of bone marrow insufficiency of varying degrees are also typical for APG, and in some cases association with aplastic anemia, less often with myelodysplastic syndrome and other hematological diseases with cerebral insufficiency. This article describes a rare clinical case of orphan disease – paroxysmal nocturnal hemoglobinuria (APG) in association with aplastic anemia.
ISSN:1819-0901
2588-0411