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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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Department of Paediatric Pneumology and Neonatology, Hannover Medical School, Carl-Neuberg-Strasse 1, OE6710, 30659 Hannover, Germany.


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.

[Indexed for MEDLINE]

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