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Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus

Thrombocytopenia is a common hematologic abnormality of childhood-onset systemic lupus erythematosus (cSLE). Although in most cases thrombocytopenia is mild, severe thrombocytopenia with bleeding complications might occur, and is further correlated with disease activity and a worse prognosis. We rep...

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Autores principales: Quintana-Ortega, Cristian, Remesal, Agustín, Vigara, Ana Pérez, Parrón-Pajares, Manuel, Bret, Montserrat, Alcobendas, Rosa, Murias, Sara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Mediterranean Journal of Rheumatology (MJR) 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10075375/
https://www.ncbi.nlm.nih.gov/pubmed/37034366
http://dx.doi.org/10.31138/mjr.33.4.459
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author Quintana-Ortega, Cristian
Remesal, Agustín
Vigara, Ana Pérez
Parrón-Pajares, Manuel
Bret, Montserrat
Alcobendas, Rosa
Murias, Sara
author_facet Quintana-Ortega, Cristian
Remesal, Agustín
Vigara, Ana Pérez
Parrón-Pajares, Manuel
Bret, Montserrat
Alcobendas, Rosa
Murias, Sara
author_sort Quintana-Ortega, Cristian
collection PubMed
description Thrombocytopenia is a common hematologic abnormality of childhood-onset systemic lupus erythematosus (cSLE). Although in most cases thrombocytopenia is mild, severe thrombocytopenia with bleeding complications might occur, and is further correlated with disease activity and a worse prognosis. We report two female patients with severe thrombocytopenia as the initial manifestation of cSLE, which were successfully treated by intensive immunosuppression including several high-dose methylprednisolone pulses and IV cyclophosphamide. Both patients were initially diagnosed with idiopathic thrombopenic purpura (ITP) refractory to conventional treatment and complicated with haemorrhagic manifestations. For this matter, patients with ITP should be assessed for the presence of ANA, anti-dsDNA antibodies, and complement levels, since they are at high risk to develop cSLE.
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spelling pubmed-100753752023-04-06 Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus Quintana-Ortega, Cristian Remesal, Agustín Vigara, Ana Pérez Parrón-Pajares, Manuel Bret, Montserrat Alcobendas, Rosa Murias, Sara Mediterr J Rheumatol Case Report Thrombocytopenia is a common hematologic abnormality of childhood-onset systemic lupus erythematosus (cSLE). Although in most cases thrombocytopenia is mild, severe thrombocytopenia with bleeding complications might occur, and is further correlated with disease activity and a worse prognosis. We report two female patients with severe thrombocytopenia as the initial manifestation of cSLE, which were successfully treated by intensive immunosuppression including several high-dose methylprednisolone pulses and IV cyclophosphamide. Both patients were initially diagnosed with idiopathic thrombopenic purpura (ITP) refractory to conventional treatment and complicated with haemorrhagic manifestations. For this matter, patients with ITP should be assessed for the presence of ANA, anti-dsDNA antibodies, and complement levels, since they are at high risk to develop cSLE. The Mediterranean Journal of Rheumatology (MJR) 2022-12-31 /pmc/articles/PMC10075375/ /pubmed/37034366 http://dx.doi.org/10.31138/mjr.33.4.459 Text en © 2022 The Mediterranean Journal of Rheumatology (MJR) https://creativecommons.org/licenses/by/4.0/This work is licensed under and Creative Commons Attribution-NonCommercial 4.0 International License.
spellingShingle Case Report
Quintana-Ortega, Cristian
Remesal, Agustín
Vigara, Ana Pérez
Parrón-Pajares, Manuel
Bret, Montserrat
Alcobendas, Rosa
Murias, Sara
Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title_full Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title_fullStr Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title_full_unstemmed Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title_short Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
title_sort severe thrombocytopenia as the main manifestation of childhood-onset systemic lupus erythematosus
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10075375/
https://www.ncbi.nlm.nih.gov/pubmed/37034366
http://dx.doi.org/10.31138/mjr.33.4.459
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