Diffuse large B-cell lymphoma in a patient with hyper-IgE syndrome: Successful treatment with risk-adapted rituximab-based immunochemotherapy
David Belada, Lukáš Smolej, Pavla Štěpánková, Pavlína Králíčková, Tomáš Freiberger
20100801
2010 Sept 1
Leuk Res
Diffuse large B-cell lymphoma in a patient with hyper-IgE syndrome: Successful treatment with risk-adapted rituximab-based immunochemotherapy
Leuk Res. 2010 Sept;34(9), David Belada, Lukáš Smolej, Pavla Štěpánková, Pavlína Králíčková, Tomáš Freiberger
1IntroductionHyper-immunoglobulin E (IgE) syndrome (HIES) or Job's syndrome is a rare primary immunodeficiency first described in 1966 [1] and characterized by eczema, Staphylococcus aureus-induced skin abscesses, recurrent pneumonias with pneumatocele formation, Candida infections, skeletal/connective tissue abnormalities and high serum level of IgE (>2000IU/ml). To date, approximately 300 cases of HIES have been published. The prevalence of HIES is lower than 1:1,000,000. Most cases are sporadic...
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