Clinical characteristics and prognostic factors of 70 patients with Sézary syndrome: a single-institutional experience at Moffitt cancer center.

Sézary syndrome (SS) is a rare and aggressive leukemic variant of cutaneous T-cell lymphoma, with a median overall survival (OS) rate of 2-4 years. Few studies have described the clinical outcome of SS patients since 2012. We retrospectively analyzed 70 patients diagnosed with SS treated at a high-volume tertiary cancer center between 2000 and 2018. Overall survival at 1 and 5 years was 84.1% and 50.7%, respectively. Univariate analyses identified older age (>65 years) and male sex as poor prognostic factors. Five patients presented with circulating large granular lymphocytic proliferation and had a favorable prognosis. Targeted therapies were effective in treating refractory/relapsed SS patients with a durable response. Therapeutic advancements and the comprehensive treatments used in a multidisciplinary clinic improved OS rates.

Hemophagocytic Lymphohistiocytosis in Malignant Hematology: Uncommon but Should Not Be Forgotten?

Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening syndrome of excessive immune activation and an inflammatory cytokine storm leading to multiple organ failure. We report our experience in a large tertiary referral center on HLH in the setting of hematologic malignancies and describe responses to therapy and outcomes. Seventeen cases of HLH were included in which the most common underlying diagnosis was aggressive lymphoma (n = 7). The median time from diagnosis of primary hematologic condition to HLH was 3.1 months. The most common presenting features were fever (n = 15), splenomegaly (n = 13), and transaminitis (n = 14). The mean serum ferritin level was 21,000 ng/mL. Fourteen patients demonstrated evidence of hemophagocytosis in bone marrow or other organs. Among all patients, 12 received etoposide, 14 received dexamethasone, and 3 received cyclosporine. Intrathecal chemotherapy was administered to 3 patients. Overall, 7 patients (41%) responded to treatment with clinical and laboratory improvement. The median overall survival (OS) from the time of HLH diagnosis and the primary hematologic diagnosis was 8.4 months and 29.5 months, respectively. The OS was better among patients with HLH with aggressive lymphomas (12 months). Response to treatment was associated with better OS. Recognition of manifestations and prompt diagnosis of HLH are crucial to initiate prompt therapy and improve outcome.

Sip-sizing behaviors in natural drinking conditions compared to instructed experimental conditions.

To understand disordered physiology, it is first necessary to determine what constitutes normal function. Liquid sip size during swallowing in healthy individuals has been investigated with varied results. Bolus size is a variable that is manipulated in both research studies and clinical swallowing assessments, so defining normal sip size has relevance in both domains. This study looked at sip size under instruction in experimental tasks and compared it to sip size in free drinking while participants were unaware that drinking was being observed. A statistically significant difference was found in water sip volume between natural drinking (mean = 16 ml) and instructed experimental drinking tasks (mean = 6.6-6.8 ml). This difference far exceeded the magnitude of sip-size variation observed between instructed drinking tasks using different stimuli and as a function of participant's gender or age group.

Tongue control for speech and swallowing in healthy younger and older subjects.

Current literature on oral motor control reports contradictory findings regarding physiological, functional and sensory changes that occur in the muscles of the tongue with normal aging. It has been suggested that the high level of activity required of tongue muscles in mastication and speech may play a role in preserving them when other skeletal muscles are more likely to show functional effects of such changes. To test whether indeed tongue movements remain unaltered in both speech and swallowing tasks as a function of aging, kinematic measures of tongue dorsum movements were taken as 21 healthy young (20-30) and older (65-74) adults performed repeated iterations of speech tasks and a sequential water swallowing task. Tongue motion was recorded using electromagnetic articulography and from these data information was extracted with respect to movement range, duration, and variability. The findings suggest that in general tongue movements for swallowing were slower and more variable than for speech, and most importantly, more variable among older than younger participants. As well, the findings show that aging does influence the nature of tongue motions, in particular by inducing a more extreme distinction in the variability of movements for speech (less variable) and swallowing (more variable) tasks.