TAILIEUCHUNG - Chapter 061. Disorders of Granulocytes and Monocytes (Part 5)

Neutrophil Abnormalities A defect in the neutrophil life cycle can lead to dysfunction and compromised host defenses. Inflammation is often depressed, and the clinical result is often recurrent with severe bacterial and fungal infections. Aphthous ulcers of mucous membranes (gray ulcers without pus) and gingivitis and periodontal disease suggest a phagocytic cell disorder. Patients with congenital phagocyte defects can have infections within the first few days of life. Skin, ear, upper and lower respiratory tract, and bone infections are common. Sepsis and meningitis are rare. In some disorders the frequency of infection is variable, and patients can go for. | Chapter 061. Disorders of Granulocytes and Monocytes Part 5 Neutrophil Abnormalities A defect in the neutrophil life cycle can lead to dysfunction and compromised host defenses. Inflammation is often depressed and the clinical result is often recurrent with severe bacterial and fungal infections. Aphthous ulcers of mucous membranes gray ulcers without pus and gingivitis and periodontal disease suggest a phagocytic cell disorder. Patients with congenital phagocyte defects can have infections within the first few days of life. Skin ear upper and lower respiratory tract and bone infections are common. Sepsis and meningitis are rare. In some disorders the frequency of infection is variable and patients can go for months or even years without major infection. Aggressive management of these congenital diseases has extended the life span of patients well beyond 30 years. Neutropenia The consequences of absent neutrophils are dramatic. Susceptibility to infectious diseases increases sharply when neutrophil counts fall below 1000 cells pL. When the absolute neutrophil count ANC band forms and mature neutrophils combined falls to 500 cells pL control of endogenous microbial flora . mouth gut is impaired when the ANC is 200 pL the inflammatory process is absent. Neutropenia can be due to depressed production increased peripheral destruction or excessive peripheral pooling. A falling neutrophil count or a significant decrease in the number of neutrophils below steady-state levels together with a failure to increase neutrophil counts in the setting of infection or other challenge requires investigation. Acute neutropenia such as that caused by cancer chemotherapy is more likely to be associated with increased risk of infection than neutropenia of long duration months to years that reverses in response to infection or carefully controlled administration of endotoxin see Laboratory Diagnosis below . Some causes of inherited and acquired neutropenia are listed in Table 61-1. .

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