AREAS COVERED: We intend to discuss the pathogenesis, diagnosis, and treatment modalities directed toward EBV-associated LPD in patients with distinct IEIs.
EXPERT OPINION: EBV-driven lymphoproliferation in IEIs presents a diagnostic and therapeutic problem that necessitates a comprehensive understanding of host-pathogen interactions, immune dysregulation, and personalized treatment approaches. A multidisciplinary approach involving immunologists, hematologists, infectious disease specialists, and geneticists is paramount to addressing the diagnostic and therapeutic challenges posed by this intriguing yet formidable clinical entity.
OBJECTIVES: QoL studies in patients with HAE have not been carried out in the Indian subcontinent. Hence, we carried out this study to assess the QoL and to identify factors associated with impaired QoL in patients with HAE.
METHODS: This was a cross-sectional observational study carried out in confirmed cases of HAE, aged >18 years, using angioedema QoL score and angioedema control test.
RESULTS: We enrolled 135 patients with HAE (aged 18-80 years) with a mean age of 40.93 years. We observed that the QoL directly correlates with angioedema control and is also affected by other factors such as gender, duration of follow-up, and the frequency of episodes. Genitalia swelling, positive family history, and presence of mortality due to HAE in the family also significantly impact the QoL of patients with HAE. In addition, patients with type 1 HAE reported a poorer QoL as compared to patients with type 2 HAE.
CONCLUSION: We report the QoL of patients with HAE from settings where none of the first-line medications are available. Results of the study suggest that disease control is the most important factor that influences the QoL.