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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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