Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.

Autor: Palvia AR; Internal Medicine, Kharghar Medicity Hospital, Navi Mumbai, IND., Damera AR; Internal Medicine, MediCiti Institute of Medical Sciences, Hyderabad, IND., Magar S; General Medicine, Kempegowda Institute of Medical Sciences, Bengaluru, IND., Nandi AR; Internal Medicine, Dr. B.R. Ambedkar Medical College & Hospital, Bengaluru, IND., Goyal M; Internal Medicine, Mayo Clinic, Rochester, USA.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2024 Jul 18; Vol. 16 (7), pp. e64866. Date of Electronic Publication: 2024 Jul 18 (Print Publication: 2024).
DOI: 10.7759/cureus.64866
Abstrakt: 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.
Competing Interests: 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.
(Copyright © 2024, Palvia et al.)
Databáze: MEDLINE