IMMUNODEFICIENY DISORDERS Flashcards

0
Q

Primary immunodeficiencies

A

B cell disorders, t cell disorders, combined b and t cells, phagocyte disorders, complement disorders

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1
Q

Recurrent infections with pyogenic bacteria indicate a immunodeficiency in

A

B cells

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2
Q

B cell disorders

A

X linked/ Bruton’s agammaglobinemia,selective IgA deficiency

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3
Q

T cell disorders

A

DiGeorge Syndrome, chronic mucocutaneous candidiasis

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4
Q

Combined B and T cell disorders

A

Severe combined immunodeficiency, wiskott aldrich syndrome, ataxia telangiectasia

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5
Q

Phagocyte disorders

A

Chronic granulomatous disease, chediak higashi syndrome, leukocyte adhesion deficiency

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6
Q

Complement disorders

A

Early- C2, C3; terminal- specific deficiency

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7
Q

Very low levels all immunoglobulins; virtual absence of B cells due to tyrosine kinase mutation

A

Bruton’s aggamoglobinemia

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8
Q

Mgmt for bruton’s

A

Pooled gamma globulin

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9
Q

Selective IgA deficiency

A

Due to failure of isotype switching and result to recurrent bacterial sinus and lung infectiob

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10
Q

Defect in B cell maturation to plasma cells

A

Common variable immunodeficiency

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11
Q

Most common form of severe antibody deficiency affecting both children and adults

A

Common variable immunodeficiency

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12
Q

Specific T cell deficiency for candida albicans

A

Chronic mucocutaneous candidiasis

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13
Q

Defect jn IL2 receptors in T cells, ADA deficiency

A

SCID

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14
Q

Inability to mount IgM response

A

Wiskott Aldrich syndrome

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15
Q

Mutation in DNA repair enzymes

A

Ataxia telangiectasia

16
Q

Lack of NADPH oxidase activity

A

Chronic granulomatous disease

17
Q

Chronic granulomatous disease presents with recurrent infections of

A

Catalase positive bacteria, A. Fumigatis

18
Q

Failure of phagolysosomal fusion, faulty microtubules impair neutrophil chemotaxis

A

Chedak higashi syndrome

19
Q

Defective adhesion (LFA 1) proteins on the surface of phagocytes

A

Leukocyte adhesion deficiency

20
Q

Most common complement defect

A

C2 deficiency

21
Q

Recurrent infections due to stap aureus

A

C3 deficiency

22
Q

Specific deficiency in C5-C9

A

Terminal complement deficiency

23
Q

Bacteremia with N. Meningitidis or N. gonorrhea

A

Terminal complement def

24
Complement responsible for opsonization
C3B
25
Complementa that are anaphylatoxin
C3a, C4a, C5a
26
Complement chemotactic to WBCs
C5a
27
Complement wc is part of the membrane attack complex
C5b-C9