TY  - JOUR
A1  - Froehlich, Matthias
A1  - Schwaneck, Eva C.
A1  - Gernert, Michael
A1  - Gadeholt, Ottar
A1  - Strunz, Patrick-Pascal
A1  - Morbach, Henner
A1  - Tony, Hans-Peter
A1  - Schmalzing, Marc
T1  - Autologous Stem Cell Transplantation in Common Variable Immunodeficiency: A Case of Successful Treatment of Severe Refractory Autoimmune Encephalitis
JF  - Frontiers in Immunology
N2  - Common variable immunodeficiency (CVID) is the most common primary immunodeficiency in adults. It is associated with hypogammaglobulinemia, recurring infections and autoimmune phenomena. Treatment includes immunoglobulin substitution and immunosuppressants. Autoimmune neurological manifestations of CVID are rare and occur predominantly as granulomatous disease. We report the case of a 35-year-old woman with CVID who developed autoimmune encephalitis as demonstrated by double cerebral biopsy. Infectious or malignant causes could be excluded. Despite intensive immunosuppressive therapy with common regimens no significant improvement could be achieved. Ultimately, an autologous hematopoietic stem cell transplantation (HSCT) was performed, resulting in lasting complete remission of the encephalitis. To our knowledge, this is the first report of refractory autoimmune phenomena in CVID treated by autologous HSCT.
KW  - common variable immunodeficiency
KW  - primary immunodeficiencies
KW  - autoimmunity
KW  - autologous stem cell transplantation
KW  - autoimmune encephalitis
Y1  - 2020
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-206972
SN  - 1664-3224
VL  - 11
IS  - 1317
ER  -