• Stephanie Cordeil, Olivier Hermine, and Arnaud Hot.
• Department of Hematology, Lyon Sud Hospital, Hospices Civils de Lyon, Pierre-Bénite, France.
• Medicine (Baltimore). 2023 Nov 24; 102 (47): e34191e34191.

RationaleKimura's disease (KD) is a rare and chronic eosinophilic related-disease, characterized by subcutaneous tissue masses, regional enlarged lymph nodes, hypereosinophilia and elevated serum IgE. KD usually affects young adults in the Asian population. In Western countries, the clinical and biological presentation of KD is often unknown, delaying the diagnosis. Therapeutic management is not standardized and despite recent advances, remission from KD can be difficult to achieve, especially in relapse situations.Patient ConcernsWe report the case of an non-Asian man with KD, initially misdiagnosed as lymphoma. We focus on his long-lasting clinical course with 20 years of recurrence despite several therapeutic lines.Diagnoses And InterventionsWe have emphasized the key points of the KD diagnostic challenge. We chose to focus on hemopathies as diagnostic traps to illustrate several overlapping features that blur frontiers with KD. With regard to treatments, lessons can be learned from the use of the therapeutic backbone, which relies on excision surgery, radiotherapy and corticosteroids.OutcomesAdvancements in KD pathogenesis have highlighted the pivotal role of Th2 lymphocytes driving eosinophil activation. Directly inspired by eosinophilic and allergic field practices, targeted therapies, such as dupilumab, provide hope for potential curative options.LessonsFinally, we propose a therapeutic plan to treat newly diagnosed KD and discuss options for relapsing entities.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

13 keywords...

hide…