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Front Med (Lausanne). 2021 Jun 16;8:678456. doi: 10.3389/fmed.2021.678456. eCollection 2021.

Immune Thrombocytopenia Revealing Enriched IgG-4 Peri-Renal Rosai-Dorfman Disease Successfully Treated with Rituximab: A Case Report and Literature Review.

Frontiers in medicine

Jerome Razanamahery, Sebastien Humbert, Jean-Francois Emile, Fleur Cohen-Aubart, Jean Fontan, Philippe Maksud, Sylvain Audia, Julien Haroche

Affiliations

  1. Internal Medicine Department and Clinical Immunology, Dijon University Hospital, Dijon, France.
  2. Internal Medicine Department, Besancon University Hospital, Besançon, France.
  3. Department of Pathology, Ambroise-Paré Hospital, Assistance-Publique Hopitaux de Paris, Paris, France.
  4. Sorbonne Université, Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Internal Medicine Department 2, National Reference Center for Histiocytosis, Paris, France.
  5. Department of Haematology, Besancon University Hospital, Besançon, France.
  6. Department of Nuclear Medicine, Pitié-Salpêtrière Hospital, Paris, France.

PMID: 34222286 PMCID: PMC8244783 DOI: 10.3389/fmed.2021.678456

Abstract

Immune thrombocytopenia (ITP) is a rare autoimmune-mediated condition characterized by isolated thrombocytopenia (<100 G/L) after exclusion of other causes. Mostly primary, it is associated with hematological malignancy, autoimmune disorders, or infection in 20% of patients. It is exceptionally described in patients with histiocytosis, mostly in children (seven patients in literature). We report a case of a 69-year-old man with ITP leading to the diagnosis of histiocytosis. At ITP's diagnosis, the patient had elevated gamma-globulins leading to computed tomography showing bilateral peri-renal infiltration. The biopsy showed enriched IgG-4 peri-renal Rosai Dorfman disease with

Copyright © 2021 Razanamahery, Humbert, Emile, Cohen-Aubart, Fontan, Maksud, Audia and Haroche.

Keywords: Erdheim Chester disease; Rosai Dorfman disease; histiocytosis; immune thrombocytopenia; rituximab

Conflict of interest statement

FC-A and JH are investigators (FC-A being the PI) of an academic study on the efficacy of cobimetinib for treating histiocytoses. The remaining authors declare that the research was conducted in the a

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