Wolanskyj-Spinner, Alexandra P

AbstractFirst‐line cytoreductive drug of choice in high risk essential thrombocythemia (ET) is currently hydroxyurea, a practice based on the results of a randomized study; second‐line drugs of choice include pegylated interferon‐α, busulfan and anagrelide. Anagrelide clinical trials were pioneered by the late Murray N. Silverstein (1928‐1998) of the Mayo Clinic whose studies led to FDA approval in March 1997. The current study represents a retrospective examination of the potential impact of anagrelide therapy on survival and disease complications in ET. 1076 patients with ET were considered (median age 58 years; females 63%); risk distribution, according to the international prognostic score for ET (IPSET), was 28% high, 42% intermediate, and 30% low. Overall (OS), myelofibrosis‐free (MFFS) and thrombosis‐free survival data were compared for ET patients diagnosed before and after the 1997 FDA approval date for anagrelide; a significant difference was apparent in OS (P = .006; HR 1.4, 95% CI 1.1‐1.7) and MFFS (P < .001; HR 4.2, 95% CI 2.7‐6.5), in favor of patients diagnosed prior to 1997; the difference was sustained during multivariable analysis that included IPSET. Similarly stratified survival data in polycythemia vera (n = 665) and primary myelofibrosis (n = 1282) showed no similar impact on survival (P = .3 and .17, respectively). The current study represents a retrospective analysis and suggests significantly decreased OS and MFFS in ET patients diagnosed after the FDA approval date of anagrelide. Whether or not anagrelide therapy was to blame for the worsening of OS and MFFS over time cannot be assumed and requires validation in a prospective study.

Throughout the years, scientific and personal research has led us on a historical journey to learn about the life of Beauchêne fils (Edme François Chauvot de Beauchêne), a French clinical anatomist, prosector, and surgeon responsible for introducing the disarticulated skull technique and the first citation of an intraneural cyst. This letter describes our research, travels, and conversations intended to uncover the fascinating history of Beauchêne fils and his family and our journey full of unexpected twists and turns. During a trip to Paris, we learned of a possible inspiration for the disarticulated skull technique from skulls afflicted with la teigne (Tinea corporis, ring worm) at the Musée Dupuytren. We toured the estate of his family's summer home in Paron, France, where we discovered a portrait of Beauchêne fils’ father that had been misidentified in our recent publication. We also discovered the date of death and elusive burial site of Beauchêne fils in Père Lachaise cemetery in Paris. We hope to bring to light the life and contributions of a nearly unknown figure who influenced not only research on intraneural ganglion cysts and the articular theory, but also the future of the field of anatomy. Clin. Anat. 31:1194–1198, 2018. © 2018 Wiley Periodicals, Inc.