For this update, we identified a new case statement which reported on a successful treatment of an individual with hemophilia B with inhibitors using ITI with rituximab, who reported the CD20\positive lymphocyte count was a useful marker to guide ITI therapy (Kobayashi 2015). Regarding safety issues, rituximab\related adverse reactions have been reported to include the following: sinusitis (Carcao 2006); nausea (Collins 2009; Fox 2006); dyspnea (Mateo 2006; Moschovi 2006); hypotension (Mateo 2006); urinary illness (Fox 2006); fever (Moschovi 2006); abdominal pain (Cooper 2006); and headache (Collins 2009). bibliographic databases and trial registries. Day of last search of the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Coagulopathies Tests Register: 16 February 2017. Selection criteria Randomized controlled tests and controlled medical trials investigating the effectiveness and security of rituximab for treating inhibitors in people with hemophilia. Data collection and analysis No randomized controlled tests coordinating the selection criteria were eligible for inclusion. Main results No randomized controlled tests on rituximab for treating inhibitors in people with hemophilia were recognized. Authors’ conclusions We were unable to identify any relevant Serlopitant tests on the effectiveness and security of rituximab for treating inhibitors in people with hemophilia. The research evidence available is definitely from case reports and case series. Randomized controlled tests are needed to evaluate the effectiveness and security of rituximab for this condition. However, prior to the publication of any possible Serlopitant long term randomized controlled tests, meta\analysis of case reports and case series may provide some evidence. Plain language summary Rituximab for treating inhibitors in people with inherited severe hemophilia Review query We reviewed the evidence available to see if rituximab is effective and safe when treating clotting element inhibitors in people with severe hemophilia. This is an upgrade of a previously published Cochrane Review. Background Hemophilia A and B are inherited conditions in which there is either reduced levels (or none whatsoever) of element VIII (hemophilia A) or element IX (hemophilia B) in the blood. In severe forms you will find undetectable levels of these factors (less than 0.01 international units (IU) per milliliter). People with hemophilia are at risk of bleeding events which can happen spontaneously CACNA2 or after stress or invasive medical procedures. Therefore, they need to become treated with element concentrates, either in reaction to these Serlopitant events or preventatively. Regrettably, about 30% of people with severe hemophilia A and 1% to 6% of people with severe hemophilia B can develop antibodies (inhibitors) against element VIII or element IX, because the factors are not identified by the immune system. The development of inhibitors is the main complication of hemophilia treatment, because their presence reduces or cancels out the beneficial effects of alternative therapy, making it very difficult to control bleeding. Moreover, when inhibitors are present, it is impossible to start preventative treatment with element VIII or element IX concentrates. Therefore, it is important to remove the inhibitors and allow treatment to continue successfully. The ‘off\label’ use (currently unapproved for treating people with hemophilia) of rituximab, has shown in some studies an effect on removing inhibitors in people with hemophilia. Therefore, we wanted to observe whether using rituximab was better than the standard treatment or additional treatments without rituximab, and whether it is safe, and could save these people from existence\threatening hemorrhage and huge monetary expense. Search date The evidence is definitely current to: 16 February 2017. Key results We did not find any randomized controlled trials assessing rituximab in people with severe hemophilia. Well\designed controlled tests are needed to assess the benefits and risks of using rituximab in people with hemophilia. Until controlled tests are published, only limited and low\level evidence, based on individual cases, can guideline physicians in making clinical decisions. Background For any glossary.