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Inflammatory Disorders of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
No peripheral blood findings are diagnostic of the disease: Full blood count: anemia, increased monocyte count.Serum calcium: elevated.Serum immunoglobulins: hypergammaglobulinemia.Serum ACE: elevated in about two-thirds of patients, but it is neither sensitive (sensitivity varies from 56% to 86%) nor specific. The false-positive rate in a normal population is about 2–4%. The level of serum ACE can correlate with the severity of the lung disease and the presence or absence of extrathoracic disease – but also with ACE-inhibitor therapy.
The Lymphatic/Immune System and Its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
The objective of the immune system is to identify and remove foreign invasion. The body, however, is capable of developing antibodies and sensitized lymphocytes directed against components normally present in the individual. Autoimmunity (immunity to one's self) leads to a number of conditions, depending on the tissues to which the immune system reacts. For example, systemic lupus erythematosus (SLE) is a connective tissue disorder that presents with skin eruptions, arthralgia, leukopenia, and other symptoms. Autoimmunity is generally implicated by the presence of hypergammaglobulinemia (excess of gamma globulins). Other diseases with an autoimmune component include rheumatoid arthritis and autoimmune thyroiditis.
The lymphoreticular system and bone marrow
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
This tumour usually affects adults in later life. It causes widespread lymphadenopathy, hepatosplenomegaly, and striking systemic symptoms such as fever, weight loss, haemolytic anaemia, and skin rashes. Hypergammaglobulinaemia may be a feature. The outlook is usually poor despite treatment.
Lymphocyte Immunophenotyping and CD4/CD8 Ratio in Cerebrospinal Fluid for the Diagnosis of Sarcoidosis-related Uveitis
Published in Ocular Immunology and Inflammation, 2021
Romain Paule, Laure Denis, Nicolas Chapuis, Julien Rohmer, Jérôme Hadjadj, Jonathan London, Anthony Chauvin, Clémence Bonnet, Luc Mouthon, Claire Le Jeunne, Dominique Monnet, Philippe Blanche, Antoine Brezin, Benjamin Terrier
By definition, lumbar puncture was performed for all patients, and was considered abnormal in case of cell count ≥10 cells/mm3. Lymphopenia was defined by a cell count <1.5 × 109/L and serum ACE level was considered abnormal if >1.5 the upper limit of normal (ULN) (>78 U/L). Hypergammaglobulinemia was defined by polyclonal gammaglobulins >15 g/L. Chest CT-scan was considered contributive in the presence of findings suggestive of sarcoidosis (i.e. mediastinal and/or hilar lymph nodes with short axis diameter >10 mm, and/or parenchymal lung abnormalities suggestive of sarcoidosis), or findings typical of healed tuberculosis (calcified granulomas predominantly in the upper lobes, and apical scarring). When bronchoscopy was performed, bronchial biopsies were considered positive if they showed granuloma, while the BALF was considered contributive in case of alveolar lymphocytosis suggestive of sarcoidosis (defined by a lymphocyte count >15% of the white cells count and a CD4/CD8 lymphocyte ratio >3.5).11
F508del CFTR gene mutation in patients with allergic bronchopulmonary aspergillosis
Published in Journal of Asthma, 2018
Maria N. Gamaletsou, Gemma Hayes, Chris Harris, Joanna Brock, Eavan G. Muldoon, David W. Denning
The identification of increased frequency of hypergammaglobulinemia in this population also raises significant questions. Given that a polyclonal gammaglobulin increase has been associated with a multitude of conditions, including chronic infection and inflammation, it may be that the apparent differences merely represent chronic suppurative infection in the context of bronchiectasis and airways inflammation, co-morbid conditions, or the time of sampling [20]. Nonetheless, it is possible that reduced CFTR function generates abnormal mucus secretions increasing the risk of endobronchial colonization with agents like Pseudomonas aeruginosa. This might increase predisposition to recurrent infections and a chronic proinflammatory response leading to hypergammaglobulinemia. The microbiological profiles of this cohort were not systematically studied and this may be a valuable area for future study.
Non-cirrhotic portal hypertension associated with multicentric Castleman’s disease: a case report
Published in Acta Oncologica, 2018
Ana Luísa Pinto, Marília Gomes, Maria Augusta Cipriano, Maria Letícia Ribeiro
Two years later, at age 34, the patient developed constitutional symptoms, consisting of malaise, anorexia, night sweats and weight loss, as well as epigastric pain. He weighted 70 kg, was afebrile, with normal blood pressure and heart rate. On physical examination, he had splenomegaly, but hepatomegaly, ascites and peripheral lymphadenopathy were absent. His laboratory evaluation revealed mild thrombocytopenia (118 × 109/L), elevated erythrocyte sedimentation rate (84 mm/h) and C-reactive protein (3.26 mg/dL). Liver enzymes were raised: aspartate aminotransferase 58 U/L (normal lower than 45 U/L); alanine aminotransferase 75 U/L (normal lower than 35 U/L); gamma-glutamyl transferase 237 U/L (normal lower than 55 U/L); and alkaline phosphatase 457 U/L (normal range 30–120 U/L). Polyclonal hypergammaglobulinemia and positive antinuclear antibodies (1:160) were also present. Bilirubin, lactate dehydrogenase, total proteins and albumin were normal.