CVID is the commonest serious antibody deficiency in adults. There are a number of associated complications including autoimmunity, of which immune thrombocytopenic purpura (ITP) is a relatively common example. ITP usually responds to treatment with corticosteroids, other immunosuppression, splenectomy or high dose intravenous immunologlobulin (IVIG). The management of ITP refractory to these treatments remains challenging. We report a patient with CVID who developed ITP and remained platelet transfusion dependent despite high dose IVIG and splenectomy. Treatment with rituximab resulted in normalisation of the platelet count and the patient remains transfusion independent at one year following rituximab therapy.
- common variable immunodeficiency
- idiopathic thrombocytopenic purpura