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COM MOD > Chapter 6- Immunodeficiency Syndromes > Flashcards

Chapter 6- Immunodeficiency Syndromes Flashcards

1
Q

When are primary (congenital) syndromes detected?

A

6 months - 2 years of life

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2
Q

What defects occur in primary congenital syndromes?

A

defect in innate immunity and defect in adaptive immunity

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3
Q

Defect in innate immune system in primary congenital syndromes

A

affect leukocyte functions or complement system

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4
Q

Defect in adaptive immunity in primary congenital syndromes

A

abnormalities in lymphocyte maturation or activation

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5
Q

Immunodeficiency Syndromes

A

SCID

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6
Q

What parts of the immune system are defective in SCID?

A

humoral and cell-mediated immune response

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7
Q

What do infants with SCID present with?

A

prominent thrush (oral fungi)
extensive diaper rash
failure to thrive

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8
Q

What infections are patients with SCID susceptible to? (5)

A 
candida albicans
Pneumocystis jiroveci
Pseudomonas 
CMV
varicella
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9
Q

When does death occur with SCID

A

in the first year without HSC (BMT) transplantation

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10
Q

Genetics of SCID

A

X linked or autosomal recessive

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11
Q

What occurs in X-linked Agammaglobulinemia

A

failure of B-cell precursors to develop into B cells

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12
Q

Characteristics of X-linked Agammaglobulinemia (4)

A

B cells are absent
All serum Igs are depressed
Plasma cells are absent
T cell-mediated reactions are normals

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13
Q

When does X-linked Agammaglobulinemia become apparent?

A

6 months which is when maternal Igs are depleted

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14
Q

What infections is a patient with X-linked Agammaglobulinemia susceptible to?

A

Recurrent bacterial infections of respiratory tract
Viruses in bloodstream or mucosal secretions
Giardia lamblia

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15
Q

Bacterial infections of respiratory tract with X-linked Agammaglobulinemia

A

haemophilus influenczae
streptococcus pneumoniae
staphylococcus aureus

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16
Q

Viruses in bloodstream or muscosal secretion in X-linked Agammaglobulinemia

A

enteroviruses- echovirus, poliovirus, coxsachievirus

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17
Q

What causes X-linked Agammaglobulinemia

A

breakdown of self tolerance and chronic infections

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18
Q

Treatment for X-linked Agammaglobulinemia

A

Replacement therapy with immunoglobulins (IgG)

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19
Q

What causes DiGeorge Symdrome

A

Failure of development of the 3rd and 4th pharyngeal pouches

20
Q

What cells are deficient in DiGeoge Syndrome?

A

T cells

Thymic hypoplasia

21
Q

What do the 3rd and 4th pharyngeal pouches give rise to

A

thymus, parathyroids, C cells of thyroid, ultimobrachial body

22
Q

Thymus in DiGeorge Syndrome

A

Loss of T cell-mediated immunity

Poor defense against fungal and viral infections

23
Q

Parathyroids in DiGeorge Syndrome

A

Hypocalcemia causing tetany

24
Q

Ultimobranchial body in DiGeorge Syndrome

A

Congenital defects of the heart and great vessels

25
Q

Genetics of DiGeorge Syndrome

A

22q11 deletion syndrome

26
Q

Characteristics of Hyper-IgM Syndrome

A

elevated IgM

can’t produce IgG, IgA, IgE

27
Q

Cause of Hyper-IgM Syndrome

A

defect in ability of helper T cells to deliver activating signals to B cells and macrophages

28
Q

Clinical presentation for Hyper-IgM Syndrome

A

recurrent pyogenic infections

29
Q

Patients with CD40L mutations in Hyper-IgM Syndrome

A

susceptible to Pneumocystic jiroveci pneumonia

30
Q

What occur if IgM antibodies react with blood cells?

A

autoimmune hemolytic anemia
thrombocytopenia
neutropenia

31
Q

What is Common Variable Immunodeficiency?

A

group of disorders with hypogammaglobulinemia (low Igs)

32
Q

Characteristics of Common Variable Immunodeficiency

A

have normal/near normal numbers of B cells

but can’t differentiate into plasma cells

33
Q

Difference between Common Variable Immunodeficiency and X-linked Agammaglobulinemia (3)

A

CVI has normal B cells, equal in male and female, and occurs in childhood or adolescence

34
Q

Characteristics of Isolated IgA Deficiency?

A

Extremely low levels of both serum and secretory IgA

35
Q

Symptomatic patient with Isolated IgA Deficiency

A

recurrent sinopulmonary infections and diarrhea
high frequency of respiratory tract allergy
autoimmune diseases

36
Q

Treatment for Isolated IgA Deficiency

A

transfuse IgA containing blood

37
Q

What occurs in IgA transfusion if patient has severe IgA deficiency?

A

IgA behaves like a foreign molecule and the patient can develop severe/fatal anaphylactic shock

38
Q

Systemic Immunodeficiencies

A

Wiskott-Aldrich syndrome

Ataxia telangiectasia

39
Q

Wiskott-Aldrich Genetics

A

X-linked

40
Q

Characteristics of Wiskott-Aldrich syndrome

A

thrombocytopenia, eczema, and recurrent infections leading to early death

41
Q

Treatment for Wiskott-Aldrich

A

HSC transplantation

42
Q

Ataxia telangiectasia Genetics

A

autosomal-recessive

43
Q

Characteristics of Ataxia telangiectasia

A 
abnormal gait
vascular malformations
neurologic deficits
increased incidence of tumors
immunodeficiency
44
Q

Secondary (Acquired) Immunodeficiency (8)

A 
Cancer
Diabetes
Complications of Cancer
AIDS
Malnutrition
Irradiation
Immunosuppressive therapy
Chemo
45
Q

Irradiation and Chemo mechanism treatments for cancer

A

decreased bone marrow precursors for all leukocytes

46
Q

Malnutrition (protein-calorie) malnutrition mechanism

A

metabolic derangements inhibit lymphocyte maturation and function

47
Q

Spleen Removal mechanism

A

decreased phagocytosis of microbes

COM MOD (21 decks)

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