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Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report

Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatospl...

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Autores principales: Njoum, Yumna, Abu-Hilal, Lila H, Jabbarin, Farah, Bannoura, Sami, Mtour, Sameer, Itmaizeh, Muaath
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10359060/
https://www.ncbi.nlm.nih.gov/pubmed/37484548
http://dx.doi.org/10.1093/omcr/omad078
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author Njoum, Yumna
Abu-Hilal, Lila H
Jabbarin, Farah
Bannoura, Sami
Mtour, Sameer
Itmaizeh, Muaath
author_facet Njoum, Yumna
Abu-Hilal, Lila H
Jabbarin, Farah
Bannoura, Sami
Mtour, Sameer
Itmaizeh, Muaath
author_sort Njoum, Yumna
collection PubMed
description Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatosplenomegaly and rash. After thorough investigation, RDD was diagnosed by mediastinal lymph node biopsy that revealed presence of S100 and CD68-positive cells with absence of CD1a confirming the diagnosis of RDD. Treatment involved combination of steroids and Rituximab, which proved to be highly effective. The patient had dramatic improvement and entered remission, with follow-up period of 2 years. It is important to note that although RDD is a rare disease, it causes severe complications, as evidenced by the patient's parameters. Thus, prompt diagnosis and treatment are paramount. Histological diagnosis is of great value, as it helps confirming and guiding treatment decisions. With the right treatment, patients can experience great recovery and quality of life.
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spelling pubmed-103590602023-07-21 Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report Njoum, Yumna Abu-Hilal, Lila H Jabbarin, Farah Bannoura, Sami Mtour, Sameer Itmaizeh, Muaath Oxf Med Case Reports Case Report Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatosplenomegaly and rash. After thorough investigation, RDD was diagnosed by mediastinal lymph node biopsy that revealed presence of S100 and CD68-positive cells with absence of CD1a confirming the diagnosis of RDD. Treatment involved combination of steroids and Rituximab, which proved to be highly effective. The patient had dramatic improvement and entered remission, with follow-up period of 2 years. It is important to note that although RDD is a rare disease, it causes severe complications, as evidenced by the patient's parameters. Thus, prompt diagnosis and treatment are paramount. Histological diagnosis is of great value, as it helps confirming and guiding treatment decisions. With the right treatment, patients can experience great recovery and quality of life. Oxford University Press 2023-07-18 /pmc/articles/PMC10359060/ /pubmed/37484548 http://dx.doi.org/10.1093/omcr/omad078 Text en © The Author(s) 2023. Published by Oxford University Press. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Njoum, Yumna
Abu-Hilal, Lila H
Jabbarin, Farah
Bannoura, Sami
Mtour, Sameer
Itmaizeh, Muaath
Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title_full Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title_fullStr Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title_full_unstemmed Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title_short Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report
title_sort rosai-dorfman disease presenting as autoimmune hemolytic anemia in a male child in palestine: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10359060/
https://www.ncbi.nlm.nih.gov/pubmed/37484548
http://dx.doi.org/10.1093/omcr/omad078
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