Брой 3/2026, Статии

Болест на фон Вилебранд – особености в детската и юношеската възраст

Публикувана на от admin
DOI: 10.66223/pediatria.2026.3.002

Д. Трайков1, Ат. Банчев1,2

1 Клиника по детска клинична хематология за доброкачествени заболявания при лица от 0 до 18 години, Специализирана болница за активно лечение на хематологични заболявания – ЕАД, София
2 Експертен център за редки анемии и вродени коагулопатии, Специализирана болница за активно лечение на хематологични заболявания – ЕАД, София

Резюме

Болестта на фон Вилебранд е най-честото наследствено нарушение на кръвосъсирването в детска възраст, с популационна честота около 1%, но значително по-ниска честота на клинично изявените и нуждаещи се от лечение случаи. Обзорът разглежда специфичните особености на заболяването при деца и юноши, като акцентира върху причините за забавена диагноза – лек мукокутанен фенотип, ограничени хемостатични предизвикателства в ранното детство, флуктуиращи нива на фактора на фон Вилебранд, влияние на стрес, възпаление, физическо натоварване, възраст и кръвна група.

Представени са основните патофизиологични механизми и съвременната класификация на заболяването – тип 1, тип 2 с подтиповете 2A, 2B, 2M и 2N, както и редкият тип 3. Разгледани са възрастово-специфичните клинични прояви: кървене при имунизации, дентална ерупция и малки хирургични интервенции при кърмачета и малки деца; епистаксис, лесно образуване на синини и постоперативно кървене в училищна възраст, тежко менструално кървене като ключов диагностичен прозорец при девойки.

Диагностичният подход изисква внимателна клинична оценка, използване на валидирани инструменти за оценка на кървенето и повторно лабораторно изследване в спокойно, базово състояние. Лечението включва десмопресин, заместителна терапия с VWF-съдържащи концентрати и антифибринолитици, най-често транексамова киселина. При девойки с тежко менструално кървене е необходим мултидисциплинарен подход с участие на хематолог, детски гинеколог и първична медицинска помощ.

Ключови думи: болест на фон Вилебранд, деца, юноши, тежко менструално кървене, десмопресин, транексамова киселина, фактор на фон Вилебранд

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Адрес за кореспонденция:

Доц. д-р Атанас Банчев, дм,

Клиника по детска клинична хематология, СБАЛХЗ,

Бул. “Климент Охридски“ 1а, София 1000. 

atanas.banchev@hematology.bg