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Successful treatment for patient with Erdheim-Chester syndrome

https://doi.org/10.33667/2078-5631-2019-3-28(403)-9-17

Abstract

Erdheim-Chester disease (ECD) is a rare systemic disease with an unknown etiology, which is characterized by infiltration of the organs CD68+, CD1a by non-langerhans cell histiocytes and occupies an intermediate position between malignant and immune disorders. The medical literature describes a little more than 750 sick peoplearound the world, and the 17 with a lesion of the bronchial tree. The largest study on this pathology includes only 53 patients. The article presents a case of personal experience of successful treatment of a patient with a morphologically established diagnosis of ECD, which differs from the standard options both in the clinical picture and in the approach to treatment. The purpose of the publication is differential diagnosis between malignant neoplasms of the skin or bronchi and ECD.

About the Authors

R. V. Orlova
Saint Petersburg State University; City Clinical Oncology Centre
Russian Federation

Saint Petersburg



N. Yu. Antimonik
City Clinical Oncology Centre
Russian Federation

Saint Petersburg



D. S. Pasekov
First St. Petersburg State Medical University named after Acad. I.P. Pavlov
Russian Federation

Saint Petersburg



O. A. Nagornaya
City Clinical Oncology Centre
Russian Federation

Saint Petersburg



T. F. Savostyanov
City Clinical Oncology Centre
Russian Federation

Saint Petersburg



N. V. Alyoshina
City Clinical Oncology Centre
Russian Federation

Saint Petersburg



A. S. Levina
City Clinical Oncology Centre
Russian Federation

Saint Petersburg



References

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2. Arnaud L., Gorochov G., Charlotte F. et al. / Systemic perturbation of cytokine and chemokine networks in Erdheim-Chester disease: a single-center series of 37 patients / Blood, 2011, 117: 2783–2790.

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4. Diamond E., Dagna L., Hyman D. et al. / Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease / Blood, 2014 Jul 24; 124 (4): 483–492.

5. Diamond E., Subbiah V., Lockhart A. et al. / Vemurafenib for BRAF V600 — Mutant Erdheim-Chester Disease and Langerhans Cell Histiocytosis: Analysis of Data From the Histology-Independent, Phase 2, Open-label VE-BASKET Study / JAMA Oncol. 2018 Mar 1; 4 (3): 384–388.

6. Mazor R., Manevich-Mazor M., Shoenfeld Y. et al. / Erdheim-Chester Disease: a comprehensive review of the literature / Orphanet Journal of Rare Diseases,2018, 38: 137.

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8. Shamburek RD, Brewer HB Jr, Gochuico BR/Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease / Am J Med Sci. 2001 Jan; 321 (1): 66–75.


Review

For citations:


Orlova R.V., Antimonik N.Yu., Pasekov D.S., Nagornaya O.A., Savostyanov T.F., Alyoshina N.V., Levina A.S. Successful treatment for patient with Erdheim-Chester syndrome. Medical alphabet. 2019;3(28):9-17. (In Russ.) https://doi.org/10.33667/2078-5631-2019-3-28(403)-9-17

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ISSN 2078-5631 (Print)
ISSN 2949-2807 (Online)