PracticeUpdate: Conference Series - EHA 2018

HLH Patients Who Survive First Critical Period May Have Low Relapse Risk Hepatomegaly andmacrophage activation syndromemay signal relatively better prognosis

A lthough the prognosis is poor for adults with hemophagocytic syndrome (HLH, hemo- phagocytic lymphohistiocytosis), patients who do live beyond the first critical period may expect survival with a low relapse risk, according to new research findings presented at EHA 2018. In their abstract, the research team, led by Rafał Machowicz, MD, PhD, with the Department of Hematology, Oncology and Internal Diseases at the Medical University of Warsaw, stated that newly diagnosed HLH adult patients with hepatomegaly and (MAS) may have a relatively better prognosis.

MAS is a potentially life-threatening complication of rheumatic disease in childhood. For reasons that remain unclear, the disease occurs much more frequently in individuals with systemic juvenile idiopathic arthritis and in those with adult-on- set Still disease. High fever, lymphadenopathy, hepatosplenomegaly variably associated with hemorrhages, and signs of liver, central nervous system and kidney involvement are the common Rituximab Yields Durable Response in AdultsWith Persistent or Chronic Immune Thrombocytopenia No unexpected long-termcomplications recorded after 12months of follow-up A fter treatment with rituximab (RTX), a durable response has been demonstrated in more than 30% of after 5 to 7 years of follow-up. A large nationwide prospective registry was set up in France in 2010 that included 248 adult patients with ITP (ClinicalTrials.Gov: NCT1101295). " Interestingly, antithrombin III and ESR were also significant in univariate analysis, but low number of available results precluded them from being tested in multivariate analysis. "

secondary ITP or who had received a previous course of RTX were excluded. A prospective and periodic assessment of the safety and the efficacy of RTX were recorded through an electronic case report form. In keeping with international guidelines, complete response was defined by a platelet count > 100x10 9 /L and response by a platelet count between 30–100x10 9 /L with at least a twofold increase from baseline. One-year follow-up data have previously been pub- lished (Khellaf et al, Blood 2014). In total, there were 248 patients included in the registry (64% female, mean age at

adults with persistent or chronic immune thrombocytopenia (ITP), according to new research findings presented at EHA 2018. In addition, the research team noted in their abstract, no unexpected long-term complications were recorded after 12 months of follow-up. At present, the long-term safety and effi- cacy of RTX in treating ITP are not well known. Current data are based only on retrospective studies. The purpose of this study was to assess long-term outcomes

ITP is a bleeding disorder characterized by a low number of platelets or throm- bocytes that results in the blood clotting improperly. Excessive bleeding and bruis- ing can result. In this study, consecutive patients ≥ 18 years of age who received RTX for a diagnosis of primary ITP based on interna- tional criteria were prospectively included between 2010 and 2012. Patients with

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