A patient with acquired agammaglobulinemia was treated with plasmapheresis. The rationale for this procedure was based on the presence of a cytotoxic autoantibody with specificity for helper (TH2-) T lymphocytes. Plasmapheresis reduced the autoantibody concentration to undetectable levels, which resulted in an increase number of helper T cells. These T cells provided normal in vitro helper acti...

The authors describe two patients with Bruton-type agammaglobulinemia, their purpose being to indicate the spectrum of findings, clinical and imaging. MR in one patient revealed diffuse leptomeningeal enhancement in the brain and spine; in the second patient, a heterogeneous mass was seen that had broken through the basiocciput and displaced a cerebellar hemisphere. Awareness of the common and ...

In evaluating the therapeutic efficacy of gammaglobulin preparations in patients with agammaglobulinemia, opsonic activity of patients' sera in terms of oxygen consumption capacity of normal leukocytes is advocated as a useful index. Although the serum lgG level is a commonly used indicator, its determination alone was found out not always to reflect antibody levels in such sera. The method des...

Induction of agammaglobulinemia in Japanese quails was attempted by various procedures of bursectomy (BX) including hormonal BX (H-BX) with testosterone, surgical BX (S-BX), chemical BX (C-BX) with cyclophosphamide and irradiation with gamma-ray. Immunosuppressive effect of BX was evaluated by antibody production in response to immunization with bovine serum albumin and sheep red blood cells, b...

No clear genotype/phenotype correlations have been established in patients with X-linked agammaglobulinemia (XLA). To determine if the specific mutation in Btk might be one of the factors that influences the severity of disease or if polymorphic variants in Tec, a cytoplasmic tyrosine kinase that might substitute for Btk, could contribute to the clinical phenotype, we examined the age at diagno...

The most common of the primary immunodeficiency diseases are those that involve inadequate antibody production. The characteristic presentation of these disorders is recurrent sinopulmonary infections. An arrest in B cell development at the pre-B cell stage leads to agammaglobulinemia and an insignificant number of B cells. X-linked agammaglobulinemia is the most common of these developmental a...

INTRODUCTION Respiratory tract infections constitute the most frequent manifestation of X-linked agammaglobulinemia (XLA). There are not many papers elucidating gastrointestinal (GI) disorders, including inflammatory bowel disease (IBD), in such patients. The aim of the study was to evaluate the occurrence of gastrointestinal disorders and IBD compared to respiratory tract infections in XLA ind...

INTRODUCTION Primary immunodeficiencies comprise diseases that impair the immune system. Clinical manifestations are characterized by recurrent respiratory infections, which may be complicated by bronchiectasis, peribronchial thickening, abscesses, bullae, and pulmonary fibrosis. The aim of this study was to determine pulmonary complications in pediatric primary immunodeficiency by type. RESULT...