Макроглобулінемія Вальденстрема

24 Серпня, 2025

Макроглобулінемія Вальденстрема (МВ) – це хронічне, повільно прогресуюче захворювання крові, яке належить до групи лімфопроліферативних хвороб. Його суть – надмірне накопичення імуноглобуліну IgM в крові, що може викликати низку серйозних ускладнень.

🔍 Як проявляється?

📌 Початок – підступний

Більшість пацієнтів не мають скарг на момент виявлення хвороби — діагноз часто ставиться випадково під час рутинного аналізу крові. Проте з часом з’являються симптоми, найпоширеніші з яких:

Слабкість – 66%
Втрата апетиту – 25%
Периферична нейропатія (оніміння, поколювання в кінцівках) – 24%
Втрата ваги – 17%
Лихоманка – 15%
Феномен Рейно – 11% (може передувати іншим симптомам на роки)

У деяких випадках можуть спостерігатися:

Сплутаність свідомості
Млявість, ступор, кома
Синдром Бінга-Ніла – ураження центральної нервової системи пухлинними клітинами

⚠️ Ознаки гіперв’язкості крові

Цей стан пов’язаний із дуже високим рівнем IgM і може бути життєво небезпечним. Типові симптоми:

Носові або ясеневі кровотечі
Запаморочення, головний біль
Погіршення зору (розмитий або подвійний зір)
Зниження слуху
Спонтанні синці

🧪 Симптоми з боку інших органів

Шлунково-кишковий тракт: діарея, кровотечі, порушення всмоктування
Шкіра: пурпура, бульозні висипи, вузлики, синдром Шніцлера (хронічна кропив’янка), ліведо, акроціаноз
Очи: крововиливи в сітківку, набряк диска зору
Нервова система: атаксія, синдром Міллера-Фішера, синдром POEMS
Серце: зрідка – застійна серцева недостатність
Легені: вузлики, інфільтрати, плевральний випіт (у 3–5%)

🔬 Що бачить лікар?

У пацієнтів часто виявляють:

Гепатомегалію (збільшення печінки) – 20%
Спленомегалію (збільшення селезінки) – 19%
Лімфаденопатію (збільшення лімфовузлів) – 15%
Пурпуру – 9%
Кровоточивість – 7%

Також можуть виникати періорбітальні пухлини (навколо очей), інфільтрація слізних залоз (причина проптозу, паралічу очних м’язів).

🧬 Що спричиняє хворобу?

Надмірна продукція IgM, що змінює в’язкість крові
Пухлинні клітини, які проникають у кістковий мозок, селезінку, лімфовузли
Мутації у генах MYD88 і CXCR4 – впливають на перебіг хвороби та лікування

💊 Як лікується?

📌 Без симптомів? – лише регулярне спостереження
📌 При симптомах:

Ритуксимаб – при легкому перебігу
Комбінована хіміотерапія – при значному ураженні
Ібрутиніб – при мутаціях MYD88 або у разі неефективності іншого лікування
Плазмаферез – при вираженому синдромі гіперв’язкості
Трансплантація стовбурових клітин – у важких або рецидивних випадках

📉 Прогноз

Захворювання вважається невиліковним, але повільно прогресує.
Середня тривалість життя – понад 6 років, а з новими методами лікування цей показник продовжує зростати.

📌 Висновок

Макроглобулінемія Вальденстрема – рідкісна, але потенційно контрольована хвороба. Своєчасна діагностика, правильне лікування та спостереження – ключ до якості життя пацієнта. При будь-яких незрозумілих симптомах – варто звернутись до гематолога.

🔔 Хочете дізнатися більше або пройти обстеження? Звертайтеся до наших фахівців у відділення гематології.

https://emedicine.medscape.com/article/207097-overview

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