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
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

e64866

Informations 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.

Auteurs

Aadi R Palvia (AR)

Internal Medicine, Kharghar Medicity Hospital, Navi Mumbai, IND.

Abhiram Rao Damera (AR)

Internal Medicine, MediCiti Institute of Medical Sciences, Hyderabad, IND.

Shikha Magar (S)

General Medicine, Kempegowda Institute of Medical Sciences, Bengaluru, IND.

Akshay Rahul Nandi (AR)

Internal Medicine, Dr. B.R. Ambedkar Medical College & Hospital, Bengaluru, IND.

Mayank Goyal (M)

Internal Medicine, Mayo Clinic, Rochester, USA.

Classifications MeSH