Summary Background Clinical and histological diagnosis of Sézary syndrome (SS) and mycosis fungoides (MF) is challenging in clinical routine. Objectives We investigated five blood markers previously described for SS (T‐plastin, Twist, KIR3DL2, NKp46 and Tox) in a prospective validation cohort of patients. Methods We included 447 patients in this study and 107 patients were followed up for prognosis. The markers were analysed by reverse transcriptase quantitative real‐time polymerase chain reaction (RT‐qPCR) on peripheral blood leucocytes and CD4+ T cells in a cohort of consecutive patients with early MF, erythrodermic MF and SS and compared with patients presenting with benign inflammatory dermatoses (BID) and erythrodermic BID. The markers were assessed in parallel to gold standard values such as CD4/CD8 ratio, loss of CD7 and CD26 membrane expression and CD4 absolute values. Sensitivity and specificity were analysed by receiver operator characteristic curves. The prognostic value of selected markers was analysed on a subset of patients. This study was conducted in one centre. Results We defined cut‐off values for each marker. T‐plastin, Twist and KIR3DL2 had the best validity. SS may be overrepresented. The combination of T‐plastin and Twist was able to differentiate between erythrodermic MF or BID and SS. The additional analysis of KIR3DL2 may be useful to predict the prognosis. Conclusions We propose T‐plastin, Twist and KIR3DL2 measured by RT‐qPCR as new diagnostic markers for Sézary syndrome.

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Patients with critical illness have disrupted circadian rhythms, which can lead to increased morbidity, mortality and length of intensive care unit stay. Light intensity within the intensive care unit influences the circadian rhythm and may therefore impact on patient outcome. We performed an observational single-centre pilot study monitoring nocturnal light exposure of intensive care unit patients between November and December 2016. As there are currently no medical guidance on recommended light levels, we audited our findings against building regulation standards. The median light intensity was 1.5 lux, which is below the 20 lux standards; however, there were significant outliers. There was positive correlation between patient illness severity based on SOFA score and maximum lux (R = 0.45, P = 0.026); however, there was no relationship between patient illness severity and median lux exposure (R = 0.23, P = 0.28). As illness severity increased so did the time spent greater than 20 lux (R = 0.59, P = 0.0021), and the individual occasions where lux breached the 20 lux limit (R = 0.52, P = 0.009). There was no relationship between illness severity of neighbouring patients and maximum lux (R = -0.11, P = 0.69) or neighbouring illness severity and median lux (R = -0.04, P = 0.87). This preliminary work will form the basis of future projects, including national guidance and evaluating the impact of environmental light on patient-centred outcomes.

Methods: S55746/BCL201 monotherapy is currently being tested in an international, first-in-human, open-label, non-randomized, doseescalation study in patients (pts) ≥18 years with relapsed or refractory B-cell NHL including mantle cell lymphoma (MCL), marginal zone lymphoma (MZL), follicular lymphoma (FL), small lymphocytic lymphoma (SLL), and diffuse large B-cell lymphoma (DLBCL). Primary objectives are to evaluate safety and establish the recommended phase 2 dose; secondary objectives include pharmacokinetics (PK), food interaction on PK, pharmacodynamics, and preliminary activity. S55746/BCL201 is administered once daily in fasting pts continuously over 21-day cycles until progressive disease (PD) or unacceptable toxicity. Pts could receive 50 to 2000 mg S55746/BCL201 according to a modified version of the continual reassessment method for dose allocation.Results: As of 9 March 2017, 37 NHL pts (median age 64 years, range 24-85) were dosed up to 1300 mg with a median duration on treatment of 42 days. NHL subtypes were DLBCL (68%), MCL (16%), FL (8%), and MZL (8%). Median number of prior regimens was 5 (range 2-15). Preliminary PK results showed that exposure increases linearly with some interindividual variability. Most common adverse events (AEs) include asthenia (n = 6), vomiting (n = 6) and nausea (n = 5).The most frequent (≥3 pts) grade ≥ 3 AEs were lymphopenia (n = 4), anemia (n = 3), and disease progression (n = 3). AEs possibly related to study drug were reported in 5 pts, the most frequent being nausea patients. Updated results up to 10 mg/kg will be presented at the meeting.

Erdheim–Chester disease is a rare multisystemic non-Langerhans cell histiocytosis presenting 95% with skeletal lesions. Erdheim–Chester disease is due to mutations in the RAS-MEK-ERK pathway where 50% are due to BRAF-V600E mutations. Typical histopathological, clinical, and radiologic features are necessary for the diagnosis of Erdheim–Chester disease. Prognosis depends on the extent of the systemic involvement, and central nervous system involvement has a poorer outcome. We present a 30-year-old Moroccan woman with diabetes insipidus, bone marrow, and asymmetrical axial osteolytic bone lesions. Biopsies were consistent with Erdheim–Chester disease. Despite no treatment, the patient has demonstrated clinical improvement.