idiopathic delayed immunoglobulin deficiency
Introduction
Introduction to idiopathic delayed immunoglobulin deficiency Idiopathic late-onset immunoglobulin deficiency (idiopathiclate-onsetIgdeficiency), also known as primary acquired agammaglobulinemia or a variable unclassified antibody-deficient disease, is a primary humoral deficiency disease. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: thymoma, vitiligo, thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia
Cause
Idiopathic delayed immunoglobulin deficiency etiology
Cause:
The reason is unknown, there is no obvious genetic tendency, and there are reports of several people in the same family who are suffering from this disease.
Pathogenesis
The pathogenesis is still unclear. Molecular genetic studies suggest that this disease and IgA deficiency may be associated with the same susceptibility gene, the complement 4A gene (C4A) located in the MHC class III region of chromosome 6.
Prevention
Idiopathic delayed immunoglobulin deficiency prevention
Strengthen care and nutrition to improve the patient's resistance and immunity, prevent infection should be isolated, and minimize contact with pathogens.
Complication
Idiopathic delayed immunoglobulin deficiency complications Complications thymoma vitiligo thrombotic thrombocytopenic purpura autoimmune hemolytic anemia
Patients are often prone to autoimmune diseases (about 22%), such as SLE, vitiligo, thrombocytopenic purpura, pernicious anemia and autoimmune hemolytic anemia, some patients can be complicated by thymoma.
Symptom
Symptoms of idiopathic late-onset immunoglobulin deficiency Common symptoms Malignant anemia repeatedly infected with gastric acid deficiency immune hemolytic immunodeficiency fat globulin globulin abnormality
Most people are 10 to 30 years old. They are found in adults. 45% of patients are diagnosed before the age of 21, and are equivalent to men and women. The family often has autoimmune diseases or immunoglobulin abnormalities.
Clinically, it is highly susceptible to purulent bacteria. Repeated bacterial infections such as influenza bacillus, pneumococci, hemolytic streptococcus and staphylococcus aureus often cause sinusitis, bronchitis, otitis media and pneumonia. Long-term morbidity can lead to chronic Progressive bronchiectasis, stomatitis and diarrhea-like syndrome (Sprue-1ike syndrome) is more common, most patients have diarrhea, fatty spasm, and sometimes protein loss enteropathy and malabsorption, some patients can apply no valley After improving the diet or avoiding milk, patients are often prone to autoimmune diseases (about 22%), such as SLE, vitiligo, thrombocytopenic purpura, pernicious anemia and autoimmune hemolytic anemia. 90% of patients have diarrhea, often with fatty sputum, gastric acid deficiency and giardiasis; 28% have bronchiectasis and splenomegaly; 12% have thyroid disease; 6% have keratitis and pernicious anemia, lymphatic reticulum The incidence of systematic malignant tumors is also high, such as gastric cancer, colon cancer and rectal lymphoma, especially in those aged 50-60 years.
Another feature is that there are often unexplained granulomas, no cheese-like necrosis, no microbes found in the lesions, often involving the skin, lungs, spleen and liver, and corticosteroids are effective.
Examine
Examination of idiopathic delayed immunoglobulin deficiency
Immunological examination: some patients may have thymoma complicated, T cells in normal blood are normal, delayed skin test is weak, B cell number is normal or increased, but B cell function is defective, such as antigen can stimulate B cells to increase Large, dividing, forming rough endoplasmic reticulum and synthetic -globulin, but not secreted, serum IgG, IgM and IgA are much lower than normal, IgG content is 0.1-2.0g/L, most patients have normal IgD, inoculating bacteria There is almost no antibody reaction after the seedling.
Histopathological examination: Lymphocytes lacked plasma cells, and lymphoid follicles were obviously proliferated, but lacked viability. Duodelia dinoflagellates were often found in duodenal biopsy, and treatment with metronidazole showed improvement.
Diagnosis
Diagnosis and differentiation of idiopathic delayed immunoglobulin deficiency
Most people are 10 to 30 years old. They are found in adults. 45% of the patients are diagnosed before the age of 21, and the male and female patients are quite similar. The family often has autoimmune diseases or immunoglobulin abnormalities. The clinical manifestations are highly susceptible to purulent bacteria. Sense, repeated bacterial infections such as influenza bacillus, pneumococcus, hemolytic streptococcus and staphylococcus aureus, often recurrent sinusitis, bronchitis, otitis media and pneumonia, long-term morbidity can lead to chronic progressive bronchiectasis, stomatitis and diarrhea Syndrome (Sprue-1ike syndrome) is more common, most patients have diarrhea, fatty sputum, sometimes protein loss enteropathy and malabsorption, some patients can be improved by applying gluten-free diet or avoiding milk It is often prone to unexplained granuloma, no cheese-like necrosis, no microbes found in the lesion, often involving the skin, lungs, spleen and liver. Corticosteroid treatment is effective. After inoculation, there is almost no antibody reaction, delayed onset. The skin test response is weak, histopathological examination of lymph nodes lacks plasma cells, and lymphoid follicles are obviously proliferated, but lack Force, duodenal biopsies often found Lanbo Gu lamblia, metronidazole treatment to improve visibility.
