Tonsillar granuloma associated with hypogammaglobulinemia

Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of CD27(+)memory B cells (30.3%) and IgD(-)IgM(-)CD27(+)switched memory B cells (7.2%; normal range 6.5-29.2%) were normal. Percentage of activated CD21(low)B cells was high (6.6%; normal range 0.6-3.5%). IgG (3.5 g/L; normal range 6.77-15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 mu g/ml after immunization with Pneumovax(R). The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.Peer reviewe

Tonsillar granuloma associated with hypogammaglobulinemia

Background Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of CD27(+)memory B cells (30.3%) and IgD(-)IgM(-)CD27(+)switched memory B cells (7.2%; normal range 6.5-29.2%) were normal. Percentage of activated CD21(low)B cells was high (6.6%; normal range 0.6-3.5%). IgG (3.5 g/L; normal range 6.77-15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 mu g/ml after immunization with Pneumovax(R). The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.Peer reviewe

Histopathological features in the progression of idiopathic pulmonary fibrosis/usual interstitial pneumonia with special emphasis on the redox modulating enzymes of the human lung

Abstract Interstitial lung diseases (ILD), including interstitial pneumonias (IP), represent disorders with variable degrees of parenchymal inflammation and/or fibrosis offer an ideal model to investigate the histopathological features in relation to the course of these diseases. The most common IP is idiopathic pulmonary fibrosis (IPF) with the histological pattern of usual interstitial pneumonia (UIP) exhibiting the histological hallmark of fibroblast foci (FF). Surgical lung biopsy (SLB) is not usually needed for diagnosis of IPF, but the lung biopsy samples taken by SLB confers the diagnosis in atypical cases. The safety of SLB in IPF/UIP has been a controversial issue. The acute exacerbation occasionally occurs during the course of IPF/UIP, but pathological features related to this event are poorly understood. Recent studies suggest that one important determinant in the pathogenesis of ILDs, as in IPF, is oxidant stress and an imbalance of the redox-state in the lung. Thiol containing redox-regulated proteins which paticipate in the antioxidant defence of the lung include thiorexin (Trx) and gamma-glutamylcysteine synthetase (γGCS), also called glutamate-cysteine ligase (GLCL), the rate-limiting enzyme of glutathione (GSH) synthesis. The goal of this research was to evaluate the safety of SLB and the relationships between the histological findings and the course of IPF/UIP, and to investigate the above mentioned defense mechanisms in a variety of ILDs by means of immmunohistochemical analyses, Western Blotting and immunoelectronmicroscopy. No deaths occurred in the following 30 days after 34 video-assisted thoracoscopic lung biopsy (VATS). The number of FF in the lung sample predicted the survival, but it was not associated with acute exacerbation of IPF/UIP before death. Diffuse alveolar damage was a common feature in autopsy samples. The studied redox regulated defense enzymes were expressed in bronchial epithelium, metaplastic alveolar epithelium and alveolar macrophages, but the fibrotic areas generally showed no expression. In IPF/UIP VATS is a safe diagnostic method and counting the number of FF represents a reproducible and reliable method for predicting patient survival. Alterations in the redox regulated defense enzymes further point to the importance of oxidant burden in the fibrotic lung

Variability in host plant chemistry : behavioural responses and life-history parameters of the colorado potato beetle (Leptinotarsa decemlineata)

v2007o

Tonsillar granuloma associated with hypogammaglobulinemia

Abstract Background: Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted. Case presentation: A 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of CD27+ memory B cells (30.3%) and IgD−IgM−CD27+ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated CD21low B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with Pneumovax®. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab. Conclusions: Tonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies