Rapamycin reduces splenomegaly and lymphadenopathy in Common Variable Immunodeficiency ( CVID ) : A
نویسنده
چکیده
A 54 year old female was hospitalised with anti-microbial-sensitive H. influenza pneumonia, her first major infection. Past medical history included prior motor vehicle accident, 160 pack year history of smoking and a recent diagnosis of chronic obstructive pulmonary disease (COPD). Medications on admission were Budesonide-Efomoterol, terbinafine and bupropion. There was no history of allergy, recent travel, sick contacts, recreational drug use or pets in the home. The patient was treated with 1.2g of IV Benzylpenicillin QID and a weaning dose of oral prednisone beginning at 50mg PO OD. She failed to clinically improve by Day 10 prompting further investigations.
منابع مشابه
Characterization of common variable immunodeficiency: identification of a subset of patients with distinctive immunophenotypic and clinical features.
The peripheral blood lymphocyte surface markers and clinical features of 38 patients with common variable immunodeficiency (CVID) were assessed. These studies identified a subset of CVID consisting of 14 of the 38 patients with a distinctive T-cell immunophenotype and clinical findings. The phenotypic changes were characterized by an abnormally low CD4/CD8 ratio (less than or equal to 0.9) due ...
متن کاملA Girl with Autoimmune Cytopenias, Nonmalignant Lymphadenopathy, and Recurrent Infections
We describe a girl, now 9 years of age, with chronic idiopathic thrombocytopenic purpura, persistent nonmalignant lymphadenopathy, splenomegaly, recurrent infections, and autoimmune hemolytic anemia. Her symptoms partly fit the definitions of both autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency disorders (CVIDs). Genetic analysis showed no abnormalities in th...
متن کاملIntravenous Immunoglobulin Monotherapy for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and ...
متن کاملAltered fraction of regulatory B and T cells is correlated with autoimmune phenomena and splenomegaly in patients with CVID.
Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency disease, leading to recurrent bacterial airway infections and often also autoimmune complications. To shed light on the regulatory lymphocytes from these patients, we analyzed the levels of regulatory B (pro-B10) cell and regulatory T (Treg) cell subpopulations in PBMCs from twenty-six patients diagnosed with CV...
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 کامل