PracticeUpdate: Conference Series - EHA 2018

31 (12%) patients died (median age: 80 [IQR, 71–84] years) after a median time of 30 (IQR, 14–54) months after the first RTX infusion, corresponding to a mor- tality rate of 2.4 (CI 95%, 1.7–3.4) for 100 patient-years. Deaths were mainly related to infections (n=6), malignancies (n=5), or bleeding (n=4), the researchers stated. Among the adverse effects, only 24 AE ≥ grade 3 were possibly related to RTX. This comprised 10 (4%) infections and 10 (4%) events related to RTX infusion. Four patients (2%) died (3 from infectious origin, 1 from an unexplained cause). Gammaglobulin levels were not system- atically monitored in the study. Among the 142 (57%) patients from whom data was available, 6 (2%) patients developed a hypogammaglobulinemia <5 g/L during follow-up. used. This database is affiliated with the Polish Adult Leukemia Group (PALG). Data were retrospectively analyzed, and a risk factor analysis was made by Cox regression. Factors were analyzed in univariable analysis, and factors with P < .06 and n > 82 were retained in the multivariable model. Sixty-two percent (53/86) of participant data came from men. The median age was 38 years (18–82). Median survival reached 144 days, but 35% of patients died within one month. Among the 29 patients who survived more than one year, two died due to HLH relapse. The longest observation exceeded 10 years. There were 34 patients with malignancy-as- sociated HLH, 20with infection-associated HLH, and 14 with HLH associated with autoimmune. In 18 patients, a triggering factor could not be identified. MAS syndrome, red blood cell count, and hypertriglyceridemia (> 265mg/dL - HLH- 2004 criterion cut-off) were determined to be positive prognostic factors. As well, a trend for hepatomegaly was observed (P = .057). “Interestingly, antithrombin III and ESR were also significant in univari- ate analysis, but low number of available results precluded them from being tested in multivariate analysis,” the investigators noted in their abstract. Of the four analyzed parameters in the multivariate analysis, only MAS and hepa- tomegaly retained the prognostic factor status. www.practiceupdate.com/c70104

characterizations of MAS. The disease may lead to multiple organ failure. HLH, a rare syndrome of fatal hyperin- flammation, occurs when a cytokine storm leads to bone marrow failure and death. Historically it was primarily diagnosed in the pediatric population, but with raising awareness, data from adults are emerg- ing, the research team noted. “Little is ITP diagnosis: 51 ± 20 years). Of all patients, 102 (41%) had persistent ITP and 146 (59%) had chronic ITP at the time RTX was first administered. Ten percent of these regis- try patients were splenectomized. After the first RTX infusion, the median fol- low-up time was 69 (IQR, 55–79] months, with a follow-up ≥ 60 months for 177 (71%) patients. At the last follow-up visit, 77 (31%) patients had a lasting response (70 CR; 7 R), stated the research team, led by Samuel Deshayes, MD, with the Department of Internal Medicine at the Centre Hospitalier Universitaire de Caen in France. Among the 177 patients with a follow-up ≥ 60 months, 50 (28%) had a lasting response (46 CR; 4 R). According to National Cancer Institute Common Terminology Criteria, 34 (14%) grade 3 or 4 infections were observed, but only 10 (4%) of these occurred within the 12 months

known about prognostic factors in HLH, especially in adult patients,” they wrote. The intent of this study was to identify prognostic factors for HLH among one of the largest cohorts of European adult patients with the disease. Data available from 86 adult (≥ 18 years of age) patients that form the HLH in Adults Database was following the last RTX infusion, including 1 pneumocystis pneumonia and 1 asper- gillosis sinusitis. There were no cases of progressive multifocal encephalopathy observed. Malignancies, however, were found in 24 (10%) patients and occurred at a median age of 71 (62–79) years of age after a median of 48 (39–62) months from RTX (incidence rate of 1.4 [CI 95%, 1.1–2.5] for 100 patient-years, similar to that observed in the French general popula- tion. No over-representation of any type of malignancy was found. The research team recorded 47 adverse effects related directly to the RTX infu- sion. This included 22 (9%) patients who developed or exacerbated another auto-immune disease; 21 (8%) patients who had cardiovascular complications; and 16 (6%) who experienced at least 1 venous thromboembolic event. In all,

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EHA 2018 • PRACTICEUPDATE CONFERENCE SERIES 15