The role of nuclear medicine in the diagnosis of Erdheim–chester disease. A series of clinical observations

A. S. Krylov; Крылов А. С.; A. D. Ryzhkov; Рыжков А. Д.; A. A. Odzharova; Оджарова А. А.; Ya. A. Shchipakhina; Щипахина Я. А.; E. E. Stanyakina; Станякина Е. Е.; A. A. Martinovich; Мартинович А. А.; K. D. Ilkaev; Илькаев К. Д.; A. M. Stroganova; Строганова А. М.; S. L. Dranko; Дранко С. Л.; O. P. Bliznyukovv; Близнюков О. П.; A. V. Kuzin; Кузин А. В.; P. A. Zeynalova; Зейналова П. А.; A. A. Semenova; Семенова А. А.; M. B. Dolgushin; Долгушин М. Б.

doi:10.17650/1818-8346-2020-15-1-28-39

The role of nuclear medicine in the diagnosis of Erdheim–chester disease. A series of clinical observations

Authors: Krylov A.S.¹, Ryzhkov A.D.¹, Odzharova A.A.¹, Shchipakhina Y.A.¹, Stanyakina E.E.¹, Martinovich A.A.¹, Ilkaev K.D.¹, Stroganova A.M.¹, Dranko S.L.¹, Bliznyukovv O.P.¹, Kuzin A.V.², Zeynalova P.A.¹, Semenova A.A.¹, Dolgushin M.B.¹
Affiliations:
1. N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
2. Russian Medical Academy of Continuing Professional Education, Ministry of Health of Russia
Issue: Vol 15, No 1 (2020)
Pages: 28-39
Section: DIAGNOSIS AND TREATMENT OF HEMOBLASTOSES
Published: 18.04.2020
URL: https://oncohematology.abvpress.ru/ongm/article/view/403
DOI: https://doi.org/10.17650/1818-8346-2020-15-1-28-39
ID: 403

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Abstract

Erdheim–Chester disease (ECD) is a rare disease with a poor prognosis. The first two cases were reported by Jakob Erdheim and William Chester in 1930. The etiology and disease incidence are unknown. One of the main components of this disorder is a chronic uncontrollable inflammation. In 2016 ECD was classified as histiocytic neoplasm by the World Health Organization and was categorized as “tumors of histiocytes and dendritic cells”. More than half of patients testing positive for the BRAF mutation. The final diagnosis is made on the basis of histological finding. There are radiological markers indicating a possible association with the disease. This is a specific, almost pathognomonic scintigraphic picture of the skeleton, “coated” aorta, “hairy” kidney patterns on computer tomography. There is a Erdheim–Chester Disease Global Alliance (ECDGA), which try to unite and provide with information about diagnostics and treatment of this rare disease both patients and doctors. Today, there are 571 registered patients and only one from Russia. We demonstrate three clinical cases of patients with newly diagnosed ECD and the role of nuclear medicine methods in assessing the prevalence of this disease.

Keywords

Erdheim–Chester disease, single-photon emission computed tomography combined with computed tomography, positron emission tomography combined with computed tomography, rare clinical case

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The role of nuclear medicine in the diagnosis of Erdheim–chester disease. A series of clinical observations

Full Text

Abstract

Keywords

About the authors

References

Supplementary files