This paper describes the first case of common variable immunodeficiency (CVID) and AA amyloidosis. A recently treated tuberculosis, and chronic inflammation induced by frequent respiratory tract infections, were thought to be responsible for the amyloidosis. No other reason for this condition could be detected. Although T cell dysfunction in some CVID patients has been reported, pulmonary tuberculosis is quite rare with this condition. Bacterial or viral agents or evidence in favour of intestinal tuberculosis, which would explain this patient's recurrent diarrhea, were not found. In this case, the response of the attacks of diarrhea to metranidazole and the histologic observation of extensive intestinal amyloid deposition, which is known to decrease intestinal motility, made us conclude that the diarrhea was associated with bacterial overgrowth. In this report, we discuss the association of CVID and tuberculosis to secondary amyloidosis and recurrent diarrhea.
Tuberculosis/*complications ; Male ; Humans ; Diarrhea/etiology ; Common Variable Immunodeficiency/*complications ; Amyloidosis/*etiology/pathology ; Adult

Adolescent ; Common Variable Immunodeficiency ; complications ; Female ; Humans ; Lung Diseases, Interstitial ; etiology ; pathology ; physiopathology ; Pneumonia ; etiology ; pathology ; physiopathology

Antiviral Agents ; therapeutic use ; Common Variable Immunodeficiency ; complications ; Epstein-Barr Virus Infections ; complications ; Humans ; Interferon-alpha ; therapeutic use ; Lymphoma ; etiology ; pathology ; therapy ; Pathology, Clinical ; methods ; trends ; Prognosis ; Zidovudine ; therapeutic use

We herein present the case of a 55-year-old woman with a previous history of malignancies--uterine adenocarcinoma, basal cell carcinoma (which occurred twice consecutively), recurrent respiratory infections due to common variable immunodeficiency (CVID), and systemic granulomatous disease diagnosed at a later age. The patient suffered from diffuse large B cell lymphoma (DLBCL), which was successfully treated with R-CHOP chemotherapy, and continued with immunoglobulin supplementation. The patient was free of lymphoma and infectious complications for over 20 months despite her persistent immunodeficiency, but eventually developed colorectal adenocarcinoma. To the best of our knowledge, this is the first reported case of CVID associated with multiple solid tumours and DLBCL.
Adenocarcinoma ; etiology ; Carcinoma, Basal Cell ; etiology ; Common Variable Immunodeficiency ; complications ; diagnosis ; therapy ; Fatal Outcome ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; etiology ; Middle Aged ; Neoplasms, Multiple Primary ; etiology ; pathology ; therapy ; Respiratory Tract Infections ; etiology ; Skin Neoplasms ; etiology ; Uterine Neoplasms ; etiology

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
Adolescent ; Bronchiectasis/etiology ; Common Variable Immunodeficiency/complications/*diagnosis/drug therapy ; Cyclosporine/therapeutic use ; Drug Therapy, Combination ; Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Injections, Intravenous ; Kidney/pathology ; Male ; Pneumonia/etiology ; Proteinuria/etiology ; Steroids/therapeutic use

No abstract available.
Adrenal Cortex Hormones/*therapeutic use ; Azathioprine/*therapeutic use ; Biopsy ; Chronic Disease ; Colonoscopy ; Common Variable Immunodeficiency/complications/diagnosis/*drug therapy ; Diarrhea/diagnosis/*drug therapy/etiology ; Drug Therapy, Combination ; Female ; Humans ; Immunoglobulins, Intravenous/*therapeutic use ; Prednisone/*therapeutic use ; Treatment Outcome ; Weight Loss/drug effects ; Young Adult