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Review
. 2020 Aug;42(6):e491-e493.
doi: 10.1097/MPH.0000000000001662.

A Case of Langerhans Cell Histiocytosis With Multifocal, Single-System GI Tract Involvement and Literature Review

Moran Gotesman  1 , Ruth Getachew  2 , Sonia Morales  3 , Kenneth M Zangwill  3 , George Gershman  4 , Steven Lee  5 , Joseph L Lasky 3rd  1 , Eduard H Panosyan  1
Affiliations
Review

A Case of Langerhans Cell Histiocytosis With Multifocal, Single-System GI Tract Involvement and Literature Review

Moran Gotesman et al. J Pediatr Hematol Oncol. 2020 Aug.

Abstract

Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder, characterized by the pathologic clonal proliferation and accumulation of immature Langerhans cells within organs. Multiple organ systems can be affected, resulting in a spectrum of clinical manifestations. Isolated gastrointestinal involvement in LCH is rare and usually presents in childhood as a multisystem disease and usually has poor outcomes. We describe a 20-year-old Hispanic female with multifocal, single-system gastrointestinal LCH. Initially diagnosed from a CD1a, S100, and CD207 (Langerin) positive appendix tissue after an appendectomy and confirmed multifocal with an endoscopy. She had a full clinical and endoscopic resolution of disease with cytarabine therapy.

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References

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