OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press48910.17650/1818-8346-2021-16-3-74-82DIAGNOSIS AND TREATMENT OF MULTIPLE MYELOДИАГНОСТИКА И ЛЕЧЕНИЕ МНОЖЕСТВЕННОЙ МИЕЛОМЫDifficulties in diagnosis of primary AL-amyloidosisТрудности в диагностике первичного AL-амилоидозаhttps://orcid.org/0000-0002-1008-5007KhyshovaV. A.ХышоваВ. А.
Russian Federation

Viktoriya A. Khyshova

4 Novyy Zykovskiy Proezd, Moscow 125167

Виктория Александровна Хышова

125167 Москва, Новый Зыковский проезд, 4

viktoria2102@icloud.comhttps://orcid.org/0000-0001-5440-4340RekhtinaI. G.РехтинаИ. Г.
Russian Federation

4 Novyy Zykovskiy Proezd, Moscow 125167

125167 Москва, Новый Зыковский проезд, 4

https://orcid.org/0000-0003-4142-171XFirsovaM. V.ФирсоваМ. В.
Russian Federation

4 Novyy Zykovskiy Proezd, Moscow 125167

125167 Москва, Новый Зыковский проезд, 4

https://orcid.org/0000-0002-4966-8146MendeleevaL. P.МенделееваЛ. П.
Russian Federation

4 Novyy Zykovskiy Proezd, Moscow 125167

125167 Москва, Новый Зыковский проезд, 4

National Medical Research Center for Hematology, Ministry of Health of RussiaФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России1009202116374820909202109092021https://oncohematology.abvpress.ru/ongm/article/view/489

Objective of the study: analysis of AL-amyloidosis (AL-A) diagnostics in real clinical practice and to determine the main approaches for the earlier detection of this disease.

Materials and methods. A retrospective analysis of medical records of 34 patients with newly diagnosed AL-A.

Results. The median time from first symptoms appearance to the diagnosis was more than 2 years. Most often, the pathological process in AL-A involves the kidneys, heart and gastrointestinal tract; moreover, at the time of diagnosis, most patients already have an injury of 2 or more organs. In half of the patients, a biopsy of the damaging organ was performed to verify the diagnosis; according to our data, histological examination of “easily accessible” locus not less informative. Symptoms characteristic of amyloidosis, such as periorbital purpura or macroglossia, are observed in a small part of patients and refer to late manifestations of the disease.

Conclusion. The versatility of AL-A manifestations leads to a late diagnosis, which affects overall survival. The main signs were highlighted that should alert doctors in relation to this pathology.

Цель исследования – проанализировать этап диагностики AL-амилоидоза (AL-А) в реальной клинической практике и наметить основные подходы для более раннего выявления этого заболевания.

Материалы и методы. Проведен ретроспективный анализ медицинской документации 34 пациентов с впервые диагностированным AL-А.

Результаты. Медиана времени от момента появления первых симптомов до постановки диагноза занимает более 2 лет. Наиболее часто в патологический процесс при AL-А вовлекаются почки, сердце и желудочно-кишечный тракт, причем на момент установки диагноза у абсолютного большинства пациентов уже имеется поражение 2 органов и более. Более чем половине больных для верификации диагноза выполнялась биопсия пораженного органа, по нашим данным, гистологическое исследование «легкодоступных» локусов не менее информативно. Характерные для амилоидоза симптомы, такие как периорбитальная пурпура или макроглоссия, наблюдаются у небольшой части больных и относятся к поздним проявлениям заболевания.

Заключение. Многогранность проявлений AL-А приводит к поздней диагностике этого заболевания, что сказывается на общей выживаемости. Были выделены основные признаки, которые должны насторожить врачей в отношении данной патологии.

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