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. OrlovaRussian Federation
Saint Petersburg
N. Yu. Antimonik
Russian Federation
Saint Petersburg
D. S. Pasekov
Russian Federation
Saint Petersburg
O. A. Nagornaya
Russian Federation
Saint Petersburg
T. F. Savostyanov
Russian Federation
Saint Petersburg
N. V. Alyoshina
Russian Federation
Saint Petersburg
A. S. Levina
Russian Federation
Saint Petersburg
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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