دورية أكاديمية
Immune Thrombocytopenia Revealing Enriched IgG-4 Peri-Renal Rosai-Dorfman Disease Successfully Treated with Rituximab: A Case Report and Literature Review.
| العنوان: | Immune Thrombocytopenia Revealing Enriched IgG-4 Peri-Renal Rosai-Dorfman Disease Successfully Treated with Rituximab: A Case Report and Literature Review. |
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| المؤلفون: | Jerome Razanamahery, Sebastien Humbert, Jean-Francois Emile, Fleur Cohen-Aubart, Jean Fontan, Philippe Maksud, Sylvain Audia, Julien Haroche |
| المصدر: | Frontiers in Medicine, Vol 8 (2021) |
| بيانات النشر: | Frontiers Media S.A. |
| سنة النشر: | 2021 |
| المجموعة: | Directory of Open Access Journals: DOAJ Articles |
| مصطلحات موضوعية: | immune thrombocytopenia, histiocytosis, Rosai Dorfman disease, Erdheim Chester disease, rituximab, Medicine (General), R5-920 |
| الوصف: | 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 MAP2K1 mutation, although peri-renal infiltration is highly suggestive of Erdheim-Chester disease. This overlapping association was described in men with mutation in MAP2K1 gene. Macrophages are implicated in the pathophysiology of ITP in multiple ways, notably by the phagocytosis of opsonized platelets and their function of antigen-presenting cells able to stimulate autoreactive T cells. Histiocytic cells derivate from monocyte-macrophage lineage. Activation of macrophages in active histiocytosis is responsible for consequential platelet destruction in ITP associated histiocytosis. Finally, this case highlights a rare presentation of ITP revealing histiocytosis, both being efficiently treated with rituximab. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 2296-858X |
| العلاقة: | https://www.frontiersin.org/articles/10.3389/fmed.2021.678456/fullTest; https://doaj.org/toc/2296-858XTest; https://doaj.org/article/c7a4b2b0b8c94049a430823ca39a67c6Test |
| DOI: | 10.3389/fmed.2021.678456 |
| الإتاحة: | https://doi.org/10.3389/fmed.2021.678456Test https://doaj.org/article/c7a4b2b0b8c94049a430823ca39a67c6Test |
| رقم الانضمام: | edsbas.8755C377 |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 2296858X |
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| DOI: | 10.3389/fmed.2021.678456 |