Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.
acute pericardial effusion
autoimmune hemolytic anemia (aiha)
coombs positive hemolysis
direct anti-globulin test
evans’ syndrome
immune thrombocytopenia (itp)
immune thrombocytopenia purpura
immune-mediated hemolysis
intravenous immunoglobulins (ivig)
prednisolone acetate
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Jul 2024
Jul 2024
Historique:
accepted:
18
07
2024
medline:
19
8
2024
pubmed:
19
8
2024
entrez:
19
8
2024
Statut:
epublish
Résumé
Evans syndrome (ES) is characterized by a combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Immune dysregulation, which results in the development of antibodies against blood cells, is its defining feature. ES being a diagnosis of exclusion requires a thorough workup to rule out other probable illnesses like lymphoproliferative diseases and systemic lupus erythematosus (SLE). We present the case of a 38-year-old male who experienced shortness of breath, chest discomfort, and generalized weakness. His medical history included recurrent anemia, thrombocytopenia, and pulmonary tuberculosis in remission. Hemolysis, thrombocytopenia, and a large pericardial effusion were discovered during the physical examination and investigations. An initial treatment strategy that included pericardiocentesis was performed. In combination with AIHA and ITP, the clinical and laboratory findings strongly suggested ES, which improved with prednisolone therapy. First-line treatments consist of corticosteroids and intravenous immunoglobulin; refractory cases may also require rituximab, thrombopoietin receptor antagonists, and sirolimus. Achieving remission and lowering relapse rates need careful patient monitoring and customized treatment programs.
Identifiants
pubmed: 39156320
doi: 10.7759/cureus.64866
pmc: PMC11330688
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Pagination
e64866Informations de copyright
Copyright © 2024, Palvia et al.
Déclaration de conflit d'intérêts
Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.