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Letter to the Editor Reply to Commentary on "A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement"
Hyun Jung Lee, MD, Tae Min Kim, MD, PhD
Cancer Research and Treatment : Official Journal of Korean Cancer Association 2012;44(4):280-280.
DOI: https://doi.org/10.4143/crt.2012.44.4.280
Published online: December 31, 2012

Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

Correspondence: Tae Min Kim, MD. Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Korea.
Tel: +82-2-2072-3559, Fax: +82-2-764-2199, gabriel9@snu.ac.kr
• Received: December 4, 2012   • Accepted: December 10, 2012

Copyright © 2012 by the Korean Cancer Association

This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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See the letter "Commentary on "A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement"" on page 279.
We express our thanks to Dr. Cavoli for interest in our case [1] and shared experience of your case. A 60-year-old female with Erdheim-Chester disease (ECD) suffered from azotemia with bilateral hydronephrosis within 2 years of initial diagnosis. Author regarded a metformin-associated lactic acidosis combined with azotemia as the cause of her presentations. Although nearly 10% of cases with metformin-associated lactic acidosis were met for all three criteria (i.e., arterial pH<7.35, blood lactate>5 mmol/L, and detectable plasma metformin concentration) [2], plasma metformin concentration was not given in this patient. In addition, fever, abdominal pain, and leukocytosis raised a possibility of urinary tract infection accompanied by azotemia.
Regardless of causality, a wait-and-see policy was possible after the correction of azotemia in this patient. Similarly, our patient did not any specific treatment for ECD until August 2012 (more than 4 years from initial detection of left renal mass) and did not any genito-urinary symptoms [3]. Therefore, a wait-and-see policy might be acceptable for asymptomatic ECD patients. Considering that interferon-α appeared to be effective against bilateral hydronephrosis [4], its response might be important to design a treatment strategy in this patient. Recently, 13 (54%) of 24 ECD patients harbored BRAF V600E mutation [5] and the identification of BRAF V600E mutation would provide a theoretical rationale for the use of BRAF inhibitors in patients with BRAF V600E-positive ECD with any symptoms or disease progression.
  • 1. Li Cavoli G. Commentary on "A case of Erdheim-Chester disease with asymptomatic renal involvement". Cancer Res Treat. 2012;44:279.ArticlePubMedPMCPDF
  • 2. Kajbaf F, Lalau JD. The criteria for metformin-associated lactic acidosis: the quality of reporting in a large pharmacovigilance database. Diabet Med. 2012;9. 21[Epub]. http://dx.doi.org/10.1111/dme.12017 Article
  • 3. Lee HJ, Lee KY, Shin DY, Lee YG, Choi SY, Moon KC, et al. A case of Erdheim-Chester disease with asymptomatic renal involvement. Cancer Res Treat. 2012;44:146–150. PMID: 22802754ArticlePubMedPMCPDF
  • 4. Haroche J, Amoura Z, Trad SG, Wechsler B, Cluzel P, Grenier PA, et al. Variability in the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of involvement: results in eight patients. Arthritis Rheum. 2006;54:3330–3336. PMID: 17009306ArticlePubMed
  • 5. Haroche J, Charlotte F, Arnaud L, von Deimling A, Helias-Rodzewicz Z, Hervier B, et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. Blood. 2012;120:2700–2703. PMID: 22879539ArticlePubMed

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    • Enfermedad de Erdheim-Chester: fractura patológica de húmero izquierdo
      Luis Gómez, Camilo Soto, Felipe Criollo, Óscar Messa, Ricardo Romo, Irene Patrón
      Revista Colombiana de Cancerología.2014; 18(3): 143.     CrossRef

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