Abstract | INTRODUCTION: CASE PRESENTATION: We here present a case of a 28-year-old woman of Turkish origin with progressive, disseminated, partly mass-forming lymphoplasmacellular infiltration (CD3+ and CD138+ cells) of the brain in association with Evans syndrome. No other central nervous system disorder could be identified on neuropathological evaluation. Although treatment with rituximab was effective to normalize erythrocyte and thrombocyte levels in her peripheral blood, it failed to dampen the inflammation in her central nervous system or prevent clinical progression. Initiation of treatment with cyclophosphamide resulted in stabilization of her central nervous system inflammation and the disease course. CONCLUSIONS: The complex immune dysregulation resulting in the antibody-mediated pathologies that can be regarded as the cause of both lymphoplasmacellular encephalitis and Evans syndrome renders this association to be of clinical relevance for both neurologists and hematologists. Our experience also sheds light on the effectiveness of different treatments for both disorders and we advise clinicians to take a closer look when encountering a combination of peripheral blood diseases with affection of the central nervous system.
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Authors | Ole Jan Simon, Tanja Kuhlmann, Stefan Bittner, Carsten Müller-Tidow, Jochen Weigt, Heinz Wiendl, Sven G Meuth |
Journal | Journal of medical case reports
(J Med Case Rep)
Vol. 7
Pg. 262
(Dec 03 2013)
ISSN: 1752-1947 [Print] England |
PMID | 24299473
(Publication Type: Journal Article)
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