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Primary and secondary immunodeficiency states
Terms in this set (29)
What are the primary immunodeficiencies?
Innate mechanism deficiencies and adaptive system dificiencies such as B, T, and stem cell deficiencies.
What are some deficiencies of Innate mechanisms?
Phagocytic cell defects and complement system deficiencies
What are some causes of phagocytic dysfunction?
Extrinsic factors, defects in metabolic pathways and results in recurrent pyogenic infections.
What are some examples of defects in extrinsic factors?
Deficiencies in antibodies, complement components, or lymphokines which activate phagocytic cells.
What are some defects in the metabolic pathway that can cause phagocytic dysfunction?
Reduced or absent levels of G-6-PD, myeloperoxidase and alkaline phosphatase ALP and Low levels of lysosomal enzymes.
What are the diseases caused by phagocytic dysfunction?
Chronic granulomatous disease, Chediak-Higashi disease, Myeloperoxidase deficiency, Lazy leukocyte syndrome, Cancer, Immunosuppressive therapy and glucose-6-phosphatase dehydrogenase deficiency.
Which disease is a X-linked or autosomal recessive
Disorder of oxidative metabolism
Defect in H2O2 production
Phagocyte ingests bacteria but does not kill
Intracellular survival of organisms results in granuloma?
Chronic granulomatous disease
Which disease Lack of myeloperoxidase in azurophilic granules
Mild to moderate defect in bacterial killing
Marked defect in fungal killing
Patient usually healthy
Frequency of infection only increased in diabetics?
Which disease has Abnormal granulation of neutrophils
Low levels of lysosomal enzymes
Which diseases has Defective locomotion or leukocyte immobility?
Lazy Leukocyte Syndrome
What are the Laboratory Assays for Phagocytic Function?
Neutrophils usually evaluated but also may evaluate monocytes/macrophages
Evaluate function by determining
and performing diagnostic testing for chronic granulomatous disease.
What are the determining factors when evaluating phagocytic function?
General metabolic integrity
Ability for chemotaxis
Ability to ingest particles
What test is used to diagnose chronic chronic granulomatous disease?
Nitroblue tetrazolium (NBT) Test
Defective neutrophils do not reduce NBT dye. Normal patients-NBT precipitated as dark blue salt in neutrophils during phagocytosis. In CGD patients-no color change
Which deficiency of the innate mechanism has defects in control proteins and deficiencies of individual complement components?
Complement System Deficiencies
What are some deficiencies of adaptive immunity?
B cell deficiencies
T cell deficiencies
Stem cell deficiencies
Which deficiency cause Bruton's agammaglobulinemia and IgA deficiency?
B cell Deficiencies
Which disease is an X-linked defect usually in males
B cells absent from bone marrow and lymphoid tissues
Deficiency or absence of peripheral blood B cells
B cells present are unresponsive to T cells
Deficiency of all classes of immunoglobulins
Normal number of T cells?
What disease has clinical findings involving Problem presents at 6 months when maternal Igs disappear
Recurrent, life-threatening pyogenic infections
Fungal infections usually not a problem
Small adenoids and tonsils?
Which disease is Most common B cell disorder
1:400 to 1:800 persons
Markedly reduced or total absence of IgA in serum and secretions
Deficiency results in increased susceptibility to bacterial infections?
Which deficiency causes DiGeorge Syndrome
Acquired Immunodeficiency Syndrome?
T cell deficiency
Which disease is the Absence of thymus and parathyroid glands at birth
Marked deficiency in T cell numbers?
Which disease has clinical findings involving Wide-set, downward slanting eyes
Low-set notched ears
Death often by age of 2 years due to infection?
Which disease involves Absent T cell function
Normal numbers but defect in B cell function
Normal T helper : T suppressor ratio
Causes progressively severe, recurrent and eventually fatal infections?
Which disease is a Autosomal recessive disorder
Hypoplastic or dysplastic formation of thymus
Defective T helper and B cells
Permanent dilatation (increase in diameter) of superficial capillaries and venules?
Which disease is the Inability to respond to polysaccharide antigens
Progressive deterioration of thymus
Decreased T cell numbers
Megakaryocytic purpura (hemorrhage into tissues)
Increased susceptibility to infection
Occurs in males
Death occurs from infection and bleeding
Survival beyond adolescence is rare?
What deficiency cause severe combined immunodeficiency and reticular dysgenesis?
Stem cell deficiency
Which disease involves an Inappropriate development of stem cells into lymphocyte precursors
Lack of T cells and B cells
Fatal hereditary disorder?
Severe combined immunodeficiency (SCID)
What are the secondary immunodeficienies
Secondary consequence of several conditions
What are the conditions that may cause secondary consequences?
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