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.
OBJECTIVE: To investigate the estimated minimal prevalence, needs, and potential interventions for the diagnosis and management of HAE in the AP.
METHODS: A structured questionnaire was distributed to representative experts from member societies of the Asia Pacific Association of Allergy, Asthma and Clinical Immunology. Patient profiles and the presence of diagnostic facilities or tests, regional and national HAE guidelines, and patient support groups were reported and compared.
RESULTS: Completed questionnaires were received from 14 representatives of 12 member countries and territories, representing 46% of the world population. Overall minimal prevalence of HAE in the AP region was 0.02/100,000 population, with significant heterogeneity across different centers. Only one-half and one-third had registered on-demand and prophylactic medications, respectively. Few had patient support groups (58%) or regional guidelines (33%), and their existence was associated with the availability of HAE-specific medications. Availability of C1-inhibitor level testing was associated with a lower age at HAE diagnosis (P = .017).
CONCLUSIONS: Hereditary angioedema in the AP differs from that in Western countries. Hereditary angioedema-specific medications were registered in only a minority of countries and territories, but those with patient support groups or regional guidelines were more likely to have better access. Asia Pacific-specific consensus and guidelines are lacking and urgently needed.
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.