Response of refractory ITP in a patient with common variable immunodeficiency (CVID) to treatment with rituximab
نویسندگان
چکیده
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.
منابع مشابه
Successful use of rituximab in refractory idiopathic thrombocytopenic purpura in a patient with common variable immunodeficiency.
Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease in patients with common variable immunodeficiency (CVID). We describe a 36-year-old woman with CVID. The clinical course of her disease was complicated by bronchiectasis, antiphospholipid antibody syndrome, and portal vein thrombosis. She developed recurrent attacks of ITP refractory to high doses of corticosteroid, intrav...
متن کاملCo-existence of Common Variable Immunodeficiency (CVID) with Idiopathic Thrombocytopenic purpura (ITP)
متن کامل
Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with Rituximab
BACKGROUND Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of comm...
متن کاملIVIG Anaphylaxis in a patient with Common variable immune deficiency disease (CVID).
Common variable immune deficiency disease (CVID) is one of the most common primary immunodeficiency disorders. It causes reduction of immunoglobulin levels and specific antibodies production and enhancement of recurrent and chronic infections risk, especially respiratory infections and gastrointestinal tracts. Here, we report a case of CVID in a 3 years old male with recurrent hospitalizations ...
متن کاملA Study of Epidemiology and Therapeutic Response of Patients with Immune Thrombocytopenic Purpura
Background: Immune thrombocytopenic purpura (ITP) is a disease characterized by decrease of the peripheral blood platelet count. The disease presents in acute and chronic forms. Because of the importance and high prevalence of ITP, it was decided to study the therapeutic response of patients with ITP. Material and Methods: A cross-sectional study was conducted at Ghaem hospital, Mashhad, Ira...
متن کامل