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Volume 33, Issue 4, December 2022



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Mediterr J Rheumatol 2022;33(4):459-64
Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus
Authors Information
  1. Paediatric Rheumatology Department, La Paz Children's Hospital, Madrid, Spain
  2. Paediatric Radiology Department, La Paz Children's Hospital, Madrid, Spain

C Quintana-Ortega, S Murias, A Remesal, M Parron-Pajares, M Bret

 
Abstract

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.


Cite this article as: Quintana-Ortega C, Remesal A, Vigara AP, Parrón-Pajares M, Bret M, Alcobendas R, Murias S. Severe Thrombocytopenia as the Main Manifestation of Childhood-Onset Systemic Lupus Erythematosus. Mediterr J Rheumatol 2022;33(4):459-64.

Article Submitted: 11 Aug 2021; Revised Form: 21 Dec 2021; Article Accepted: 15 Jan 2022; Available Online: 31 Dec 2022

https://doi.org/10.31138/mjr.33.4.459

This work is licensed under a Creative Commons Attribution 4.0 International License.

©2022 The Author(s).