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

Erdheim Chester disease Rosai Dorfman disease histiocytosis immune thrombocytopenia rituximab

Journal

Frontiers in medicine
ISSN: 2296-858X
Titre abrégé: Front Med (Lausanne)
Pays: Switzerland
ID NLM: 101648047

Informations de publication

Date de publication:
2021
Historique:
received: 09 03 2021
accepted: 13 05 2021
entrez: 5 7 2021
pubmed: 6 7 2021
medline: 6 7 2021
Statut: epublish

Résumé

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

Identifiants

pubmed: 34222286
doi: 10.3389/fmed.2021.678456
pmc: PMC8244783
doi:

Types de publication

Case Reports

Langues

eng

Pagination

678456

Informations de copyright

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

Déclaration de conflit d'intérêts

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 absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Jerome Razanamahery (J)

Internal Medicine Department and Clinical Immunology, Dijon University Hospital, Dijon, France.

Sebastien Humbert (S)

Internal Medicine Department, Besancon University Hospital, Besançon, France.

Jean-Francois Emile (JF)

Department of Pathology, Ambroise-Paré Hospital, Assistance-Publique Hopitaux de Paris, Paris, France.

Fleur Cohen-Aubart (F)

Sorbonne Université, Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Internal Medicine Department 2, National Reference Center for Histiocytosis, Paris, France.

Jean Fontan (J)

Department of Haematology, Besancon University Hospital, Besançon, France.

Philippe Maksud (P)

Department of Nuclear Medicine, Pitié-Salpêtrière Hospital, Paris, France.

Sylvain Audia (S)

Internal Medicine Department and Clinical Immunology, Dijon University Hospital, Dijon, France.

Julien Haroche (J)

Sorbonne Université, Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Internal Medicine Department 2, National Reference Center for Histiocytosis, Paris, France.

Classifications MeSH