Defects In Innate Immunity (Phagocytic Disorders) Flashcards

1
Q

What is leukocyte adhesion deficiency (LAD)?

A

A defect that results in neutrophils not being able to aggregate or bind to intercellular adhesion molecules on endothelial cells which is necessary for their egress from the vasculature into tissues

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2
Q

Pts with LAD present with a history of what?

A

Recurrent infections of the oral and genital mucosa, skin, intestinal and respiratory tracts

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3
Q

What are the clinical manifestations of LAD?

A

Delayed detachment of the umbilical cord, slow wound healing, severe bacterial infections and failure to form pus

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4
Q

What is chronic granulomatous disease (CGD)?

A

A deficiency in NADPH oxidase in phagocytes leading to their failure to from superoxide and O2 radicals
Results in defective elimination of extracellular pathogens such as bacteria and fungi

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5
Q

Pts with CGD are susceptible to infections with what pathogen?

A

Catalase positive organisms (ex. Staphylococcus)

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6
Q

What is G6PD deficiency?

A

X linked recessive and most Pts are asymptomatic
Associated with anemia bc G6PD is important in RBC metabolism
Same manifestation as CGD

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7
Q

What is Chediak Higashi syndrome (CHS)?

A

Autosomal recessive molecular defects in the structure of neutrophil granules leading to abnormalities in chemotaxis and degranulation
Pts prone to recurrent pyogenic granulomas caused by bacterial infections

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8
Q

What is commonly seen in CHS pts at age 30?

A

Wheel chair bound and death by infection

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9
Q

What is the appearance of neutrophil granules in pts with CHS?

A

Abnormal giant granules that dont contain cathepsin G or etalase

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10
Q

Describe how CHS is biphasic

A

1 phase: susceptibility to infections

2nd phase: accelerated lymphoproliferative syndrome with hepatosplenomegaly and lymphadenopathy

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11
Q

How is a diagnosis of CHS made?

A

Observation of azurophilic giant cytoplasm inclusions in RBCs, partial albinism and no NK activity

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12
Q

Which gender is CGD more common in?

A

Males

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13
Q

Both CGD and G6PD are characterized by a tendency to form what?

A

Granulomas

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