Review for 10.1186/s12876-022-02378-8 (2022)

Reviewer’s report
Title: 
Pancreatic Involvement in Erdheim-Chester Disease: A Case Report and
Review of the Literature
Version: 
0 
Date: 
15 Mar 2022
Reviewer's report:
The report is interesting. I have the following remarks. 
The Authors state that 'For patients with wild type BRAF, IFN-
α
 is still the first
line therapy, especially in developing countries'. This sentence should be
rephrased. Patients with severe wild type BRAF ECD should receive MEK
inhibitors. 
The Authors state that 'Molecular profiling of the biopsies tissue didn't find any
BRAF mutations'. Were other mutations downstream the MAPK or in related
pathways investigated in this case? 
The Authors state that 'During the arterial phase and portal phase, such lesions
showed reduced enhancement (Figure 1 e)'. What does this suggest in terms of
tissue composition of lesions? Is it compatible with xanthogranuloma? 
EUS-FNA showed no evidence of malignancy, yet what did it find? I cannot tell
from the picture, which is quite grainy and does not provide bars. In the absence
of evidence of foamy CD68+ CD1a- macrophages in the pancreas, the main
finding supporting ECD-related pancreas involvement is the fact that lesions
regressed following IFN therapy. However, were other causes of transient
pancreatitis ruled out? 
The authors fail to discuss the role of inflammation in the development of ECD
lesions. See and discuss the recent papers 'Oncogene-induced maladaptive
activation of trained immunity in the pathogenesis and treatment of Erdheim-
Chester disease. Molteni et al. Blood 2021' and 'Oncogene-induced senescence in
hematopoietic progenitors features myeloid restricted hematopoiesis, chronic
inflammation and histiocytosis. Biavasco et al. Nature Communications 2021'.
Modulation of inflammation may explain the brilliant response to treatment in
this patient. 
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