Erdheim–Chester disease with cardiac involvement. Case report

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Abstract

Erdheim–Chester disease (ECD) is a rare non-Langerhans histiocytosis with multisystem inflammatory infiltrates consistent of monocytes/macrophages, reactive microenvironment and fibrotic fields. Cardiovascular involvement is one of the most frequent manifestations of ECD that can lead to life threating complications. In this article we are reporting a clinical case of ECD with cardiac involvement in a young patient.

About the authors

Vitalii D. Latyshev

National Research Center for Hematology

Author for correspondence.
Email: LatyshevVD@gmail.com
ORCID iD: 0000-0003-0643-8807

врач-гематолог отд-ния орфанных заболеваний

Russian Federation, Moscow

Elena А. Mershina

Lomonosov Moscow State University

Email: LatyshevVD@gmail.com
ORCID iD: 0000-0002-1266-4926

канд. мед. наук, доц. каф. лучевой диагностики и лучевой терапии, зав. отд-нием рентгенодиагностики с кабинетами МРТ и КТ

Russian Federation, Moscow

Irina E. Kostina

National Research Center for Hematology

Email: LatyshevVD@gmail.com
ORCID iD: 0000-0003-4683-4118

канд. мед. наук, врач-рентгенолог, зав. отд-нием рентгенологии и компьютерной томографии

Russian Federation, Moscow

Valentin Е. Sinitsyn

Lomonosov Moscow State University

Email: LatyshevVD@gmail.com
ORCID iD: 0000-0002-5649-2193

д-р мед. наук, проф., зав. каф. лучевой диагностики и лучевой терапии фак-та фундаментальной медицины, рук. отд. лучевой диагностики университетской клиники

Russian Federation, Moscow

Elena A. Lukina

National Research Center for Hematology

Email: LatyshevVD@gmail.com
ORCID iD: 0000-0002-8774-850X

д-р мед. наук, проф., зав. отд-нием орфанных заболеваний

Russian Federation, Moscow

References

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Supplementary files

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2. Fig. 1. Localization of infiltrates in the region of the heart and large vessels in patient K. (data from CT and MRI): a – infiltrate in the region of the left atrium with compression of the pulmonary veins (CT, 2020); b – rounded infiltrate in the region of the apex of the pancreas (MRI, 2018); c – para-aortic infiltrate (CT, 2018); d–f – Infiltrates in the pulmonary artery with narrowing of the lumen of the pulmonary artery (CT and MRI, 2020); g – three-dimensional reconstruction of CT images in 2018 (zones of specific infiltration are shown in red); h – Infiltrates in the region of the mouth of the inferior vena cava (MRI, 2020).

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3. Fig. 2. Trametinib-induced periorbital edema in patient K.

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