Chapter 061. Disorders of Granulocytes and Monocytes (Part 7)

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Miscellaneous Metabolic disorders—ketoacidosis, acute renal failure, eclampsia, acute poisoning Drugs—lithium Other—metastatic carcinoma, acute hemorrhage or hemolysis Abnormal Neutrophil Function Inherited and acquired abnormalities of phagocyte function are listed in Table 61-3. The resulting diseases are best considered in terms of the functional defects of adherence, chemotaxis, and microbicidal activity. The distinguishing features of the important inherited disorders of phagocyte function are shown in Table 61-4. Table 61-3 Types of Granulocyte and Monocyte Disorders Cause of Indicated Dysfunction Function Drug-Induced Acquired Inherited Adherenceaggregation Aspirin, colchicine, alcohol, state, Neonatal Leukocyte adhesion deficiency types 1 and 2 glucocorticoids, ibuprofen, piroxicam hemodialysis Deformability ...

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Chapter 061. Disorders of Granulocytes and Monocytes (Part 7) Miscellaneous Metabolic disorders—ketoacidosis, acute renal failure, eclampsia, acute poisoning Drugs—lithium Other—metastatic carcinoma, acute hemorrhage or hemolysis Abnormal Neutrophil Function Inherited and acquired abnormalities of phagocyte function are listed in Table 61-3. The resulting diseases are best considered in terms of the functional defects of adherence, chemotaxis, and microbicidal activity. The distinguishing
features of the important inherited disorders of phagocyte function are shown in Table 61-4. Table 61-3 Types of Granulocyte and Monocyte Disorders Cause of Indicated Dysfunction Function Drug-Induced Acquired Inherited Adherence- Aspirin, Neonatal Leukocyte aggregation colchicine, alcohol, state, adhesion glucocorticoids, hemodialysis deficiency types 1 ibuprofen, piroxicam and 2 Deformability Leukemia, neonatal state, diabetes mellitus, immature neutrophils Chemokinesis- Glucocorticoids Thermal Chédiak- (high dose), auranofin, injury, Higashi syndrome,
chemotaxis colchicine (weak malignancy, neutrophil-specific effect), malnutrition, granule deficiency, phenylbutazone, periodontal hyper IgE– naproxen, disease, neonatal recurrent infection indomethacin, state, systemic (Job's) syndrome interleukin 2 lupus (in some patients), erythematosus, Down syndrome, rheumatoid α-mannosidase arthritis, diabetes deficiency, severe mellitus, sepsis, combined influenza virus immunodeficiency, infection, herpes Wiskott-Aldrich simplex virus syndrome infection, acrodermatitis enteropathica, AIDS Microbicidal Colchicine, Leukemia, Chédiak- activity cyclophosphamide, aplastic anemia, Higashi syndrome, glucocorticoids (high certain neutrophil-specific
dose), TNF-α blocking neutropenias, granule deficiency, antibodies tuftsin chronic deficiency, granulomatous thermal injury, disease, defects in sepsis, neonatal IFN-γ/IL-12 axis state, diabetes mellitus, malnutrition, AIDS Table 61-4 Inherited Disorders of Phagocyte Function: Differential Features Clinical Cellular or Diagnosis Manifestations Molecular Defects Chronic Granulomatous Diseases (70% X-linked, 30% Autosomal Recessive) Severe infections of No respiratory NBT or DHR test; skin, ears, lungs, liver, and burst due to the lack of no superoxide and H2O2
bone with catalase-positive one of four NADPH production by microorganisms such as S. oxidase subunits in neutrophils; immunoblot aureus, Burkholderia cepacia neutrophils, monocytes, for NADPH oxidase , Aspergillus spp., and eosinophils components; genetic Chromobacterium violaceum ; detection often hard to culture organism; excessive inflammation with granulomas, frequent lymph node suppuration; granulomas can obstruct GI or GU tracts; gingivitis, aphthous ulcers, seborrheic dermatitis Chédiak-Higashi Syndrome (Autosomal Recessive) Recurrent pyogenic Reduced Giant primary infections, especially with S. chemotaxis and granules in neutrophils aureus; many patients get phagolysosome fusion, and other granule-bearing lymphoma-like illness during increased respiratory cells (Wright's stain); adolescence; periodontal burst activity, defective genetic detection disease; partial egress from marrow,
oculocutaneous albinism, abnormal skin window; nystagmus, progressive defect in LYST peripheral neuropathy, mental retardation in some patients Specific Granule Deficiency (Autosomal Recessive) Recurrent infections of Abnormal Lack of secondary skin, ears, and sinopulmonary chemotaxis, impaired (specific) granules in tract; delayed wound healing; respiratory burst and neutrophils (Wright's decreased inflammation; bacterial killing, failure stain), no neutrophil- bleeding diathesis to upregulate specific granule contents chemotactic and (i.e., lactoferrin), no adhesion receptors with defensins, platelet α stimulation, defect in granule abnormality; transcription of granule genetic detection proteins; defect in C/EBPε Myeloperoxidase Deficiency (Autosomal Recessive)
Clinically normal No No peroxidase in except in patients with myeloperoxidase due to neutrophils; genetic underlying disease such as pre- and posttranslational detection diabetes mellitus; then defects candidiasis or other fungal infections Leukocyte Adhesion Deficiency Type 1: Delayed Impaired Reduced separation of umbilical cord, phagocyte adherence, phagocyte surface sustained neutrophilia, aggregation, spreading, expression of the CD18- recurrent infections of skin chemotaxis, containing integrins with and mucosa, gingivitis, phagocytosis of C3bi- monoclonal antibodies periodontal disease coated particles; against LFA-1 defective production of (CD18/CD11a), Mac-1 CD18 subunit common or CR3 (CD18/CD11b), to leukocyte integrins p150,95 (CD18/CD11c); genetic detection Type 2: Mental Impaired Reduced
retardation, short stature, phagocyte rolling along phagocyte surface Bombay (hh) blood endothelium expression of Sialyl- phenotype, recurrent Lewisx, with monoclonal infections, neutrophilia antibodies against CD15s; genetic detection Phagocyte Activation Defects (X-linked and Autosomal Recessive) NEMO deficiency: Impaired Poor in vitro mild hypohidrotic ectodermal phagocyte activiation by response to endotoxin; dysplasia; broad based IL-1, IL-18, TLR, CD40, lack of NF-κB activation; immune defect: pyogenic and TNF-α leading to genetic detection encapsulated bacteria, viruses, problems with Pneumocystis, mycobacteria; inflammation and X-linked antibody production IRAK4 deficiency: Impaired Poor in vitro susceptibility to pyogenic phagocyte activation by response to endotoxin; bacteria such as staphylococci, endotoxin through TLR lack of NF-κB activation streptococci, clostridia; and other pathways; by endotoxin; genetic
resistant to mycobacteria; TNF-α signaling detection autosomal recessive preserved Hyper IgE–Recurrent Infection Syndrome (Autosomal Dominant) (Job's Syndrome) Eczematoid or pruritic Reduced Clinical features, dermatitis, "cold" skin chemotaxis in some involving lungs, skeleton, abscesses, recurrent patients, reduced and immune system; pneumonias with S. aureus suppressor T cell activity serum IgE > 2000 IU/mL with bronchopleural fistulae and cyst formation, mild eosinophilia, mucocutaneous candidiasis, characteristic facies, restrictive lung disease, scoliosis, delayed primary dental deciduation Mycobacteria Susceptibility (Autosomal Dominant and Recessive Forms)
Severe local or Inability to kill Low or very high disseminated infections with intracellular organisms levels of IFN-γ receptor bacille Calmette-Guérin due to low IFN-γ 1; functional assays of (BCG), nontuberculous production; mutations in cytokine production and mycobacteria, salmonella, IFN-γ receptors, IL-12 response; genetic histoplasmosis, poor receptor, IL-12 p40, detection granuloma formation STAT-1, NEMO Abbreviations: GI, gastrointestinal; GU, genitourinary; NADPH, nicotinamide-adenine dinucleotide phosphate, NBT, nitroblue tetrazolium (dye test), DHR, dihydrorhodamine (oxidation test); LYST, lysosomal transport protein; C/EBPε, CCAAT/enhancer binding protein-ε; NEMO, NF-κB essential modulator; TLR, Toll-like receptor; IL, interleukin; TNF, tumor necrosis factor; IRAK4, IL-1 receptor–associated kinase protein-ε, NEMO 4; IFN, interferon.[newpage]