In common variable immunodeficiency disorders (CVID), splenectomy has been used mainly in refractory autoimmune cytopenia. The aim of the study is to explore circumstances and outcomes after therapeutic splenectomy in CVID.
We retrospectively reviewed two cases of CVID patients who underwent splenectomy.
Case 1: A 19-year-old boy born to non-consanguineous parents, followed for CVID revealed at 16 years of age, with chronic respiratory symptoms, interstitial lung disease, lymphoproliferative syndrome, and recurrent infections. Respiratory pathology has been attributed to a granulomatous lymphocytic interstitial lung disease. He had heterogeneous splenomegaly exceeding 20 cm leading to hypersplenism and refractory cytopenia. Splenectomy was done at the age of 19 years; The pathological study showed granulomatosis. The short-term evolution was favorable.
Case2: A 42-year-old woman, followed for CVID, diagnosed at age 26, in front of the family history of CVID, episodes of recurrent skin infections, ORL (sinusitis and otitis) and respiratory (recurrent pneumopathies with obliterant bronchopathy), as well as autoimmune thrombocytopenia. Biology has shown a low level of different immunoglobulins and low B-memory-lymphocytes. The patient was splenectomized for corticosteroid-dependent autoimmune thrombocytopenia. The intervention was followed by a long-term normalization of platelet count but without a significant reduction in infections after a 20-year follow-up.
Splenectomy is recommended as a therapeutic option for patients with refractory cytopenias and/or autoimmunity, even though CVID patients undergoing splenectomy may be at increased risk for infection given their intrinsic immunological defects. Future trials are needed to provide clearer guidance on the second-line management.