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Pathology 2 > Immunodeficiency > Flashcards

Immunodeficiency Flashcards

0
Q

Low levels of all immunoglobulins due to tyrosine kinase mutation

A

Bruton’s disease

B cell

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1
Q

B cell disorders

A

X Linked Hypogammaglobulinemia or Bruton’s Disease
Selective IgA deficiency
Common Variable deficiency

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2
Q

6 month old male with recurrent pyogenic bacterial (S pneumoniae and H influenzae) and enteroviral infections

A

Bruton’s disease

Pooled gamma globulin

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3
Q

Failure of isotype switching

A

Selective IgA deficiency

B cell

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4
Q

Recurrent bacterial sinus and lung infections

A

Selective IgA deficiency

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5
Q

Defect in B cell maturation

A

Common Variable Immunodeficiency

B cell

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6
Q

Most common form of severe antibody deficiency

A

CVID

Pooled gamma globulin

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7
Q

T cell disorders

A

DiGeorge

Chronic mucocutaneous candidiasis

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8
Q

Failure of development of thymus and parathyroids due to defect in 3rd and 4th pharyngeal pouches

A

DiGeorge
T cell
Transplant of fetal thymus

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9
Q

CATCH 22

A 
Cardiac TOF
Abnormal facies
Thymic aplasia
Cleft palate 
Hypocalcemia 
22q11.2 chromosomal deletion
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10
Q

Recurrent candidiasis in children

A

Chronic mucocutaneous Candidiasis

Azole

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11
Q

Combined B and T cell

A

SCID
Wiskott-Aldrich
Ataxia- Telangiectasia

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12
Q

SCID
X linked
Autosomal

A

Gamma chain/IL-2 receptor gamma

Adenosine deaminase deficiency

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13
Q

Recurrent viral, bacterial, fungal and protozoal infection at 3 months of age

A

SCID
Plastic bubble
BM transplant

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14
Q

Inability to mount IgM response

A

Wiskott Aldrich

B and T cell

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15
Q

Wiskott Aldrich

Symptomatology

A

TIE Me a WASP
Thrombocytopenia
Infections
Eczema

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16
Q

Mutations in DNA repair enzymes

IgA deficiency

A

Ataxia-Telangiectasia

B and T cell

17
Q

Ataxia
Telangiectasia
Infections before 2 yo

A

Ataxia Telangiectasia

BM transplant

18
Q

Phagocyte disorders

A

Chronic Granulomatous Disease
Chediak Higashi
Leukocyte Adhesion deficiency

19
Q

Lack of NADPH oxidase activity

A

Chronic Granulomatous Disease

Phagocyte

20
Q

Recurrent infections with catalase positive bacteria and fungi (A fumigatus)
Widespread granulomas

A

Chronic Granulomatous Disease

21
Q

Failure of phagolysosomal fusion

Faulty microtubules

A

Chediak Higashi

22
Q

Delayed separation of umbilical cord

A

Leukocyte Adhesion Deficiency

Phagocyte

23
Q

C1 deficiency leading to increased C3a and C5a

A

Hereditary angioedema

Complement

24
Q

Widespread angioedema

Fatal larngeal edema

A

Hereditary angioedema

Corticosteroids

25
Q

Most common complement defect

A

C2 deficiency

26
Q

Bacteremia with Neisseria

A

Terminal Complement Deficiency
Cannot form MAC
Vaccination

27
Q

Hemoglobinuria upon waking up

28
Q

Deficiency of decay accelerating factor

29
Q

AIDS

Screening test

A

ELISA

Anti gp120

30
Q

AIDS

Confirmatory test

A

Western blot

P24 antigen and gp41 antibodies

31
Q

AIDS

Monitoring immune status

32
Q

AIDS
Test for active replication
Marker for progression
Most sensitive test for acute HIV before seroconversion

A

HIV viral load

33
Q

CD4<500

A

M tb
HSV
Candida albicans
HHV-8

34
Q

CD4<200

A 
PCP
Toxoplasma gondii
Cryptococcus neoformans
Coccidioides immitis
C parvum
35
Q

CD4<50

A

M aviun
Histoplasma capsulatum
CMV

36
Q

Marked follicular hyperplasia with viral DNA in the nuclei of CD4 cells in the LN

A

Early AIDS

37
Q

Burnt out lymph nodes

  • follicular depletion
  • disrupted organization
38
Q 
Amyloidosis proteins
Primary
Secondary
Senile cardiac
T2-DM associated
Medullary thyroid
A 
AL from light chain
AA from acute phase reactants
Transthyretin from AF old folks
Amylin from AE endocrine
A-CAL from calcitonin
39
Q

Amyloidosis

Tissue biopsy

A

Abdominal fat aspirate

40
Q

Most common and serious form of amyloidoses

A

Renal amyloidosis

41
Q

Tapioca like granules in the splenic follicles

Map like areas in walls of splenic sinuses

A

Sago spleen
Lardaceous spleen
Amyloidosis

Pathology 2 (20 decks)

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