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Flashcards in Immunodeficiency Deck (42):
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B cell disorders

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

1

Low levels of all immunoglobulins due to tyrosine kinase mutation

Bruton's disease
B cell

2

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

Bruton's disease
Pooled gamma globulin

3

Failure of isotype switching

Selective IgA deficiency
B cell

4

Recurrent bacterial sinus and lung infections

Selective IgA deficiency

5

Defect in B cell maturation

Common Variable Immunodeficiency
B cell

6

Most common form of severe antibody deficiency

CVID
Pooled gamma globulin

7

T cell disorders

DiGeorge
Chronic mucocutaneous candidiasis

8

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

DiGeorge
T cell
Transplant of fetal thymus

9

CATCH 22

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

10

Recurrent candidiasis in children

Chronic mucocutaneous Candidiasis
Azole

11

Combined B and T cell

SCID
Wiskott-Aldrich
Ataxia- Telangiectasia

12

SCID
X linked
Autosomal

Gamma chain/IL-2 receptor gamma
Adenosine deaminase deficiency

13

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

SCID
Plastic bubble
BM transplant

14

Inability to mount IgM response

Wiskott Aldrich
B and T cell

15

Wiskott Aldrich
Symptomatology

TIE Me a WASP
Thrombocytopenia
Infections
Eczema

16

Mutations in DNA repair enzymes
IgA deficiency

Ataxia-Telangiectasia
B and T cell

17

Ataxia
Telangiectasia
Infections before 2 yo

Ataxia Telangiectasia
BM transplant

18

Phagocyte disorders

Chronic Granulomatous Disease
Chediak Higashi
Leukocyte Adhesion deficiency

19

Lack of NADPH oxidase activity

Chronic Granulomatous Disease
Phagocyte

20

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

Chronic Granulomatous Disease

21

Failure of phagolysosomal fusion
Faulty microtubules

Chediak Higashi

22

Delayed separation of umbilical cord

Leukocyte Adhesion Deficiency
Phagocyte

23

C1 deficiency leading to increased C3a and C5a

Hereditary angioedema
Complement

24

Widespread angioedema
Fatal larngeal edema

Hereditary angioedema
Corticosteroids

25

Most common complement defect

C2 deficiency

26

Bacteremia with Neisseria

Terminal Complement Deficiency
Cannot form MAC
Vaccination

27

Hemoglobinuria upon waking up

PNH

28

Deficiency of decay accelerating factor

PNH

29

AIDS
Screening test

ELISA
Anti gp120

30

AIDS
Confirmatory test

Western blot
P24 antigen and gp41 antibodies

31

AIDS
Monitoring immune status

CD4 Count

32

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

HIV viral load

33

CD4<500

M tb
HSV
Candida albicans
HHV-8

34

CD4<200

PCP
Toxoplasma gondii
Cryptococcus neoformans
Coccidioides immitis
C parvum

35

CD4<50

M aviun
Histoplasma capsulatum
CMV

36

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

Early AIDS

37

Burnt out lymph nodes
- follicular depletion
- disrupted organization

Late AIDS

38

Amyloidosis proteins
Primary
Secondary
Senile cardiac
T2-DM associated
Medullary thyroid

AL from light chain
AA from acute phase reactants
Transthyretin from AF old folks
Amylin from AE endocrine
A-CAL from calcitonin

39

Amyloidosis
Tissue biopsy

Abdominal fat aspirate

40

Most common and serious form of amyloidoses

Renal amyloidosis

41

Tapioca like granules in the splenic follicles
Map like areas in walls of splenic sinuses

Sago spleen
Lardaceous spleen
Amyloidosis