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Sunday, February 16, 2014

DISEASES OF Neutrophils

•         
Neutrophils are small cells with a nucleus with 2-5 lobes has short life span 6-8 hours.
   Function of neutrophils:
•          The prime function of neutrophils is to ingest and kill bacteria, fungi and damaged cells.
•          Neutrophils are released into the circulation in response to stimuli e.g infections and corticosteriods therapy.
•          Neutrophil leucocytosis (neutrophillia)
A rise in the number of circulating neutrophils to >10x109 occur in bacterial infections or as a result of tissue damage, pregnancy, during excersise and corticosteriod therapy.
Causes of neutrophil leucocytosis
•          Bacterial infection
•           Tissue necrosis in myocardial infarction
•          Inflammation
•          Corticosteriod therapy
•          Hematological , myelofibrosis, Leukomoid reaction, Leukoerythroblastic reaction
•          Pregnancy, excersise
•          Malignant (breast,bronchial-gastric)
•          Metabolic ,renal failure
•           Congenital, neutrophil adhesion deficiency.
•          Neutropenia and agranulocytosis
Neutropenia is defined as a circulating neutrophil count below 1.5x109/l. Absence of neutrophil is called agranulocytosis.
Causes of Neutropenia:
A- Congenital : is rare in which there is a defect in maturation and release of neutrphils from the bone marrow  (Kostman’s syndrome, cyclic neutropenia every 2-3 weeks).
•          Acquired causes
Viral infections, severe bacterial infections (typhoid)., Felty’s syndrome, immune neutropenia and pancytopenia.
•          Leukemia, myelosuppressive drugs, antiviral drugs, hypersplenism.
•          Clinical features:
Infections may be frequent when neutrophil count is below 0.5 (severe neutropenia) and may be associated with septicemia and may be associated with pneumonia and septicemia.
•          Patients presented by glazed mucositis in the mouth and ulceration.
•          Disseminated infection are usually occurred with septicemia, fungemia and deep abscesses, local infections ,mouth, perianal, skin damage and vascular catheters.
•          Treatment
Growth factors also used successfully in the treatment of chronic neutropenia.
•          Steroids and high dose intravenous immunoglobulins are used to treat patients with severe autoimmune neutropenia and recurrent infections.
•           
•          Causes of neutrophil dysfunction
A-Congenital:
•          1-Leucocyte adhesion defect: autosomal recessive disorder impaired leukocyte tissue localization,locomotion,and endocytosis.
•          2-Hyper-IgE syndrome: the syndrome characterized by very high levels of IgE,impaired neutrophil locomotion,severe eczema with frequent staphylococcal secondary infections, abscesses and fungal infections.            
•          Shwachman’s syndrome
Exocrine pancreatic insufficiency and Pyogenic infections, defects in neutrophil migration.
•          Chronic granulomatous disease
•          This is a congenital defect of neutrophil and monocyte killing.
•          Clinical features:
•          Patients have chronic suppurative granulommas or abccess affecting skin, lymph nodes, liver, lung as well as osteomyelitis.
Treatment: Antimicrobial therapy, surgical measures, regular gamma interferon can reduce the frequency of infection.
•          Acquired neutrophil dysfunction
Causes:
•          Corticosteriods therapy, affect neutrophil function and cell mediated immunity.


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