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Proteins in plasma and urine
Published in Martin Andrew Crook, Clinical Biochemistry & Metabolic Medicine, 2013
Hypogammaglobulinaemia can be defined as a decrease in plasma immunoglobulins. Total plasma protein concentration is usually low. A marked reduction in plasma immunoglobulin concentrations may be detectable as obvious hypogammaglobulinaemia in the serum electrophoretic pattern, but usually measurement of individual proteins is needed to make a diagnosis. The effects of deficiencies of individual immunoglobulins are related to their functions and distribution: IgA deficiency may be symptomless, or may be associated with recurrent, mild respiratory tract infections or intestinal diseases.IgG deficiency may result in recurrent pyogenic infections of tissue spaces, especially in the lungs and skin, by toxin-producing organisms such as staphylococci and streptococci.IgM deficiency frequently predisposes to septicaemia.
Rhinosinusitis: Definitions, Classification and Diagnosis
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Rhinosinusitis may also be a presenting or secondary feature of patients with immunodeficiencies (e.g. HIV84) or who are immunocompromised by systemic treatment for other disorders (e.g receiving immunosuppressive drugs for organ transplantation). In those cases, patients require endoscopic retrieval of mucopus where possible, as unusual organisms may be found.85 Immunodeficiency should always be considered a possibility in patients who appear not to respond to standard medical and surgical care for CRS86,87 and in cases of recurrent ARS. Common variable immune deficiency (CVID), selective IgA deficiency, IgG subclass deficiency, and specific antibody deficiency are all possible immunodeficiencies that can be detected in cases of CRS.88 Refractory cases of CRS have been shown to have low IgG in 18%, low IgA in 17% and low IgM in 5%89 with both IgA and IgG deficiencies postulated as potential contributory factors for patients failing medical therapy for CRS.90 In children, where a higher index of suspicion accompanies the onset of CRS, IgG3 subclass deficiency has been reported in association with the presence of nasal polyposis.91 Another study looking at 245 CRS patients failing prolonged antibiotic therapy found that about 8% had an IgG deficiency92 with IgG3 deficiency being implicated as the most common Ig deficiency in the wider group of patients with CRS93 and IgG2 in cases of recurrent ARS.94 Certainly there is a role for screening for immunodeficiency in CRS patients refractory to standard treatment.88,95
GATA2 Deficiency
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Diagnostic workup for GATA deficiency involves the following: Medical history review: Personal and family history of warts, mycobacterial infection, autoimmunity, chest disease, cytopenia, acute myeloid leukemia.Physical examination: For warts and other abnormalities.Laboratory tests: Blood count for monocytopenia; lymphocyte subsets for B cell and NK cell deficiency, CD4:CD8 inversion (<1·0), absence of CD1c+, CD141+ and plasmacytoid blood DC; occasional IgA or IgG deficiency; bone marrow for megakaryocyte dysplasia, hypocellularity, fibrosis; blood for lupus anticoagulant, elevated FLT3 ligand (10- to 100-fold), absent transitional B cells and CD56bright NK cells; bone marrow flow cytometry for loss of primitive multi-lymphoid progenitors (MLP), lymphoid-primed multipotent progenitors (LMPP), and reduction of granulocytic monocytic progenitors (GMP); normal CD56+ plasma cells present; lungs for diminished lung volumes and transfer factor; pulmonary infiltrates on CT; PAP on biopsy without GM-CSF antibodies.Histopathology: Bone marrow for megakaryocyte dysplasia, hypocellularity, fibrosis; tissues biopsy with special stains for mycobacteria and fungi; neoplastic lesions for HPV and herpes virus nucleic acid or antigens.Cytogenetic and molecular analyses: Monosomy 7; trisomy 8; ASXL1 mutation; GATA-2 gene deletion, mutation in codons 1–398 or intron 5 enhancer (Figure 74.3) [24,25].
Immunological Role of IgG Subclasses
Published in Immunological Investigations, 2021
Cecilia Napodano, MariaPaola Marino, Annunziata Stefanile, Krizia Pocino, Roberto Scatena, Francesca Gulli, Gian Lodovico Rapaccini, Stefano Delli Noci, Giovanna Capozio, Donato Rigante, Umberto Basile
IgG1 is the most abundant subclass and the main IgG to cross the placenta. In fact, neonatal serum levels are similar to the maternal ones, though they drop over few months following birth (Burtis et al. 2012). One of the most relevant cause of hypogammaglobulinemia can be IgG1 deficiency, due to its higher level respect to other subclasses. IgG1 deficiency is associated with increased susceptibility to recurrent infections, and respiratory tract infection is the most common manifestation of undiagnosed IgG subclass deficiency. The diagnosis of IgG deficiency is made, on average, after 7 years of frequent or severe respiratory tract infections. Adults may be diagnosed to have IgG deficiency after longer intervals of infections than observed in younger patients (Barton et al. 2019).
Gamma globulin replacement therapy in uncontrolled, severe asthma associated with humoral immunodeficiency: A series of five case reports
Published in Journal of Asthma, 2019
Angelica Tiotiu, Yves Martinet, Roger Jankowski, Philippe Devillier
Case 3. A 55-year-old woman presented with uncontrolled, nonallergic, corticosteroid-dependent asthma (prednisone 20 mg/day), diagnosed 25 years prior (FEV1 = 48% of predicted; FEV1/FVC = 0.64). Recurrent sinus and lung infections required monthly consultations, treatment with antibiotics, and an increase in the daily dose of prednisone. The patient was a former smoker (40 pack-years) and suffered from obesity (BMI = 33 kg/m2), sleep apnea syndrome (apnea–hypopnea index: 23.4/h), and gastroesophageal reflux. Although a CT scan of the chest was normal, a CT scan of the sinuses revealed maxillary sinusitis. Bacteriological tests of the bronchoalveolar lavage were positive for Streptococcus sp., Haemophilus influenza, and Haemophilus parainfluenzae. We diagnosed an IgG deficiency (5.72 g/L; normal values: 7–16 g/L) with predominant IgG2 subclass deficiency (1.57 g/L; normal values: 2.41–7 g/L). The vaccine-induced antibody responses and the B lymphocyte count were normal. After the failure of prophylactic treatment with azithromycin (250 mg every other day for 3 months), subcutaneous gamma globulin replacement therapy (10 g/week) decreased the frequency of asthma exacerbations and respiratory infections, improved asthma control (with a fall in the ACQ-7 score from 3.5 to 1.7), and enabled the replacement of oral corticosteroids with appropriate inhaled medication (beclomethasone 800 µg/day, formoterol 24 µg/day, and tiotropium 5 µg/day).
Airway disease in hematologic malignancies
Published in Expert Review of Respiratory Medicine, 2022
Ricardo J José, Burton F. Dickey, Ajay Sheshadri
The goal of therapy in bronchiectasis is to prevent infective exacerbations and to treat these rapidly and adequately. This will reduce inflammation and injury in the airways, thereby attenuating progression of the disease. Furthermore, treatment aims to reduce symptoms and maintain quality of life. Patients should be assessed by a pulmonologist with a special interest in bronchiectasis and pulmonary infections to receive a comprehensive assessment of host immunity, and to develop personalized therapeutic strategies that include airway clearance, early treatment of infections, prevention of frequent infective exacerbations using prophylactic oral or nebulized antibiotics, and vaccinations. The general management of bronchiectasis is discussed elsewhere [101,114]. Of importance in patients with hematologic malignancies is the identification of those with significant IgG deficiency, severe deficiency of switched memory B-cells, or functional antibody deficiency with poor responses to pneumococcal polysaccharide vaccination. In those with >2 exacerbations per year despite prophylactic antibiotics, administration of intravenous immunoglobulins must be considered. In the study by José et al, IgG deficiency was not associated with survival, most likely due to universal immunoglobulin replacement therapy. Monitoring of IgG and the impact of intravenous immunoglobulin (IVIG) on the incidence of infections is required to assess efficacy of treatment. In some cases, IgG levels return to normal and IVIG may no longer be required. However, an assessment of function (e.g. vaccination responses) is necessary because function may remain impaired despite normal total IgG levels. Prophylactic antibiotics usually consist of azithromycin 250 mg daily or 500 mg three times, or, if contra-indicated, doxycycline 100 mg daily. In some patients, with drug intolerances other antibiotics are used, usually guided by the sensitivity of previously identified bacteria. For example, in patients with frequent infections with Stenotropomonas maltophilia or Acinetobacter baumanii, co-trimoxazole is considered. In cases where recurrent infections/exacerbations persist, nebulized antibiotics such as colomycin or nebulized are also used, particularly when infections are frequently due to gram negative bacteria.