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Cancer Research and Treatment > Volume 44(4); 2012 > Article
Lee and Kim: Reply to Commentary on "A Case of Erdheim-Chester Disease with Asymptomatic Renal Involvement"
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.
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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
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: 22802754
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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: 17009306
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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: 22879539
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