Rare form of erdheim-chester disease presenting with isolated central skeletal lesions treated with a combination of alfa-interferon and zoledronic Acid

E N Bulycheva; V V Baykov; M I Zaraĭskiĭ; G N Salogub

doi:10.1155/2015/876752

Rare form of erdheim-chester disease presenting with isolated central skeletal lesions treated with a combination of alfa-interferon and zoledronic Acid

Case Rep Hematol. 2015:2015:876752. doi: 10.1155/2015/876752. Epub 2015 Apr 8.

Authors

E N Bulycheva¹, V V Baykov², M I Zaraĭskiĭ², G N Salogub²

Affiliations

¹ First Pavlov State Medical University of St. Petersburg, L'va Tolstogo Street, No. 6/8, Saint Petersburg 197022, Russia ; University Hospital Carl Gustav Carus, Fetscherstrasse 74, 01307 Dresden, Germany.
² First Pavlov State Medical University of St. Petersburg, L'va Tolstogo Street, No. 6/8, Saint Petersburg 197022, Russia.

Abstract

Erdheim-Chester disease (ECD) represents a clonal non-Langerhans histiocytosis, which manifests under an extensive variety of clinical symptoms. This creates a challenge for the physician, who is required to recognize and diagnose the disease in the early stages. Despite this considerable challenge, in the last decade there has been a dramatic increase in ECD diagnoses, in most part due to an increasing awareness of this rare disorder. Involvement of the axial skeleton is exclusively uncommon with no official recommendations for the treatment of the bone lesions. Here, we present a case report of a young male patient with isolated lesions of the spine, ribs, and pelvis, who was successfully treated with a combination therapy of alfa-interferon and zoledronic acid.