RESUMO
CONTEXT.: In 2022, 2 distinct guidelines for the diagnosis of myeloid neoplasms became available: the 5th edition of the World Health Organization guideline (WHO2022) solely and the International Consensus Classification (ICC). Despite major overlap, there are important differences that can have important implications. OBJECTIVE.: To explore the current opinions and diagnostic practices of hemato-oncologists and hematopathologists across the United States. DESIGN.: An online anonymous survey was created using REDCap, and a secure link was shared via email to fellowship program leaderships and via posts on social media. RESULTS.: A total of 310 responses were obtained. Only 33 of 309 respondents (10.7%) reported using solely the 2016 World Health Organization guideline to make diagnoses, whereas 167 of 309 (54%) supplemented it with other guidelines. The rest were either not sure (17; 5.5%), used WHO2022 solely (46; 14.9%), or used ICC solely (6; 1.9%). The choice of guideline was not related to region (P = .15), practice setting (P = .86), or hospital size (P = .22). More than 90% reported it is a source of confusion in clinical diagnosis, management, trial design, and other areas. CONCLUSIONS.: Overall, our study found that having 2 distinct guidelines could be a source of confusion for physicians and calls for a unified diagnostic language.
RESUMO
Extra-nodal natural killer T-cell lymphoma (ENKTL) is a rare and aggressive hematologic malignancy found in the nasal cavity and adjacent locations in 80% of cases, accounting for approximately 10% of non-Hodgkin lymphoma (NHL) and 0.4% of all cancers. Prognosis is typically poor and depends on stage, location, age, and tumor markers for targeted therapy, which is reserved for relapsed/refractory ENKTL. Of those, advanced clinical stage, higher Prognostic Index (PI), nodal involvement, Ki-67 expression, large cells, local tumor invasiveness, and circulating Epstein-Barr virus (EBV)-DNA levels are predictive of worse survival. Here, we present a rare case of a patient in remission 30 months after diagnosis of ENKTL following a sustained complete response to pembrolizumab, an immune checkpoint inhibitor targeting the programmed death-1 (PD-1) receptor.
