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USMLE REVIEW > Immunology > Flashcards

Immunology Flashcards

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USMLE® Step 1 flashcards
1
Q

Recurrent viral, fungal and protozoal infections

Hypocalcemia and Tetany

A

DiGeorge Syndrome
Thymic Aplaisa
Failure of the 3rd and 4th pharyngeal pouches to develop

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2
Q

Congenital heart defects associated with DiGeorge Syndrome?

A

Tetrology of Fallot

Truncus Arteriosis

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3
Q

Chromosomal abnormality associated with 90% of DiGeorge Syndrome

A

22q11 Deletion

*Detect with FISH

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4
Q

Recurrent Bacteria

Low levels of all immunoglobulins

A

Bruton’s agammablobulinemia

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5
Q

Defect in Bruton’s agammaglobulinemia

A

Defect in BTK
Tyrosine kinase gene
No B-cell maturation

X-linked recessive = increased in BOYS

**Disease of “B”s

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6
Q

Reccurent Sinus and lung infections

Anaphalaxis following blood transfusion

A

Selective IgA deficiency

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7
Q

Failure to thrive
Chronic Diarrhea
Thrush
Recurrent viral, bacterial, fungal, protozoal infections

A

SCID

Adenosine Deaminase Deficiency

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8
Q

2 immunodeficiencies with absence of thymic shadow?

A

DiGeorge

SCID

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9
Q

Poor smooth pursuit
Ataxia
Telangiectasia of face
Increased AFP (after 8 months of age)

A

Ataxia-telangiectasia

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10
Q

Why should pts w/ ataxia-telangiectasia avoid x-rays?

A

Defect is in ATM gene = failure to repair DNA double strand breaks and cell cycle arrest

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11
Q

Pneumonic for ATAXIA- Telangectasia

A

ATAXIA

Ataxia
Telangectasia
Acute leukemia/lymphoma increased risk
X-ray sensitivity
IgA deficiency
AFP increased
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12
Q

Thrombocytopenia and purpura
Eczema
Recurrent Pyogenic Infections

A

Wiskott-Aldrich Syndrome

WATER=
Wiskott
Aldrich
Thrombocytopenia purpura
Eczema
Recurrent Pyogenic Infections
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13
Q

Defect in Wiskott-Aldrich Syndrome?

A

Defect in WAS gene

T-cells unable to reorganize actin cytoskeleton

**X-linked recessive, more common in boys

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14
Q

Defect in Hyper IgM syndrome

A

Defective CD40L on Th cells

Can’t class switch

High IgM
Low IgG, IgA, IgE

X-linked recessive

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15
Q

Delayed separation of umbilical cord

Impaired wound healing

Absent pus formation

Neutrophilia

A

Leukocyte Adhesion Deficiency

Defect in LFA-1 integrin = CD18

*impaired migration and chemotaxis

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16
Q

Increased susceptibility to catalase + organisms?

A

Chronic Granulomatous Disease

Defect in NADPH oxidase

Can’t convert O2 to superoxide dismutase for respiratory burst in neutrophils/phagocytes

17
Q

Chronic Granulomatous Disease especially susceptible to….

A

Staph Aureus
Aspergillus
Pseudomonas

18
Q

Negative Nitroblue Tetrazolium test

A

Chronic Granulomatous Disease

Normally (+) would oxidize the dye to blue/black pigment… but can’t oxidize it because they can’t generate oxygen free radicals

19
Q

Treatment for chronic granulomatous disease

A

Prophylactic TMP-SMX and itraconazole

IFN-gamma

20
Q

Partial Albinism
Recurrent respiratory and skin infections
Neurologic Disorders

A

Chediak-Higashi Syndrome

21
Q

Defect in Chediak-Higashi

A

Defective LYST gene (lysosomal transport)

Defective phagocyte lysosomes = giant cytoplasmic granules in PMNs are diagnostic

22
Q 
Eczema
Recurrent cold abscesses (S. Aureus)
Coarse facial features
Retained primary teeth
High IgE
A

Job Syndrome = Hyper IgE syndrome

Defect in STAT3 - impaired recruitment of neutrophils to sites of infections (why abscesses are cold, no inflammation)

23
Q

Large phagosomal vesicles in neutrophils

A

Chediak Higashi

24
Q

Transplant rejection in minutes due to preformed Abs

A

Hyperacute rejection

25
Q

Transplant rejection with CD8+ T cells against donor MHCs (Abs develop after transplant)

A

Acute rejection (weeks- months)

26
Q

Transplant rejection with CD4+ T-cells

A

Chronic Rejection

Months-years

27
Q

Dermatitis
Hepatitis
Gastroenteritis
Weeks after transplant

A

Graft-vs-host disease

Grafted immunocompetent T cells proliferate in the immunocompromised host and reject host cells

*usually in bone marrow and liver transplants

USMLE REVIEW (25 decks)

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