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Common Variable Immunodeficiency in Two Adult Patients Diagnosed after Lymphoma As First Presentation.
Saturday, March 5, 2016
South Exhibit Hall H (Convention Center)
Maria G Kanariou, Sofia Tantou, Marianna Tzanoudaki, Marina Siakantari, Angelos Pefanis
Rationale: Common Variable Immunodeficiency (CVID) is a Primary Immunodeficiency with broad range of clinical manifestations; lymphomas to be one of the major causes of death in adulthood. We present two adult patients whose CVID diagnosis was performed after lymphoma disease. 

Methods: Flow Cytometry and ELISA were performed for diagnosis. 

Results: Patient A was asymptomatic to the age of 35, when he was diagnosed with B-Non-Hodgkin Lymphoma (Burkitt type). He was successfully treated with combination of anti-CD20 and CHOP. During his investigation hypogammaglobulinemia was revealed which insisted. While in complete remission, he suffered severe infections and the immune investigation shown inability to produce specific antibodies and absence of memory switching B-cells (IgD-IgM-CD27+: <0.1% on B-cells). Eight years now he is in good clinical condition with gamma-globulin (SCIG) supplementation therapy. Patient B presented at the age of middle twenties, with Hodgkin Lymphoma, and he was given ABVD chemotherapy with good response. However, five years later he underwent autologous hematopoietic stem cell transplantation (aHSCT) after high dose chemotherapy because of relapse. Afterwards, he experienced severe bacteremic pneumococcal pneumonia and his investigation revealed extremely low immunoglobulin levels, lack of specific antibodies production and almost undetectable memory switching B-cells (IgD-IgM-CD27+: <0.6% on B-cells). Patient is managed by gamma-globulin replacement therapy (SCIG) and he is asymptomatic for almost two years.

Conclusions: These cases are reported because lymphoma was the initial clinical manifestation of CVID at an older age than has been reported and both of them are well under gamma-globulin supplementation therapy. Increase awareness for CVID diagnosis is needed.