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Br J Haematol. 2010 Feb;148(3):445-8. doi: 10.1111/j.1365-2141.2009.07987.x. Epub 2009 Nov 17.

Successful treatment of autoimmune and lymphoproliferative complications of patients with intrinsic B-cell immunodeficiencies with Rituximab.

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1
Department of Paediatric Pneumology and Neonatology, Hannover Medical School, Carl-Neuberg-Strasse 1, OE6710, 30659 Hannover, Germany. Hennig.christian@mh-hannover.de

Abstract

The heterogeneous group of primary immunodeficiencies requires personalized diagnosis and therapy to acheive an optimal outcome for each patient. This was exemplified by two patients with intrinsic B-cell class-switch defects (subclass of Hyper-IgM syndromes), where lymphoproliferation and autoimmunity determined the clinical course for many years due to lack of exact diagnosis. Based on genetics or a novel functional diagnostic approach, a definite individual diagnosis was established for each patient and they started Rituximab therapy. Autoimmune phenomena and generalized lymphadenopathy disappeared and remained well controlled during the observation period (3-4 years) without adverse effects. Quality of life increased remarkably in both patients.

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