Primary Immunodeficiency (Monteleone) Flashcards
(18 cards)
1
Q
IgG, IgM, IgA, IgD, IgE half lives
A
IgG: 23 days IgM: 5 days IgA: 6 days IgD: 3 days IgE: 2.5 days
2
Q
Signs of Immunodeficiency
A
- 4+ ear infections/year
- 2+ sinus infections/year
- 2+ months antibiotics without effect
- Failure to grow/thrive (infant/kid)
- Abcesses
- Thrush/skin fungal infections
- Need for IV antibiotics
- Septicemia
- FAMILY HISTORY of primary immunodeficiency
- Unusual infections (only possible with compromised immune system)
3
Q
Suspicious Infections for T-cell Deficiency
A
- Fungal infections like pneumocystis (pneumonia), cryptococcus (meningitis)
- Herpesvirus
4
Q
Suspicious Infections for B-cell Deficiency
A
- Bacterial infections like haemophilus (pneumonia, bacteremia), streptococcus (pneumonia), Campylobacter (GI infections)
- Parasites like Giardia (GI infection)
- Enteroviruses
5
Q
Suspicious Infections for Phagocyte Deficiency
A
- Streptococcus, Burkholderia (pneumonia), Serratia (respiratory, skin, urinary infections), Norcardia (pneumonia)
- Fungal infections like aspergillus (respiratory infections)
-Neutrophil oxidative burst test to diagnose
6
Q
Suspicious Infections for Monocyte Deficiency
A
- Nontuberculosis Mycobacteria (respiratory infections)
- Salmonella (GI infection)
7
Q
Suspicious Infections for Complement Deficiency
A
-Neisseria (meningitis or gonorrhea) - since commonly killed by MACs, cant form without complement
8
Q
Primary Immunodeficiency
A
- Immune deficiency due to genetic disorder (as opposed to acquired or due to drugs)
- Commonly inherited/genetic
- Infection-prone
9
Q
Severe Combined Immunodeficiency (SCID)
A
- T-cell deficiency causing total lack of immune system
- Lack T-cells, affects B/NK cell development too
- Infections, failure to thrive
- Kids die by age 1
10
Q
DiGeorge Syndrome
A
- Impacts development of 3rd/4th pharyngeal arch organs, including heart (tetrology of fallot), thymus (so low/absent T-cells)
- Treat with bone marrow transplant
11
Q
Ataxia Telangectasia
A
- T-cell deficiency
- also defects in B-cells - low IgA//IgE
- Neurologic defects- loss of motor skills in childhood
- Telangectasia- “spiderweb” of veins
- Susceptible to infections
12
Q
Wiskott-Aldrich Syndrome
A
- T-cell deficiency (also affects B-cells)
- small platelets
- thrombocytopenia (low platelets)
- petechiae, bruising, bleeding
- Eczema
- Infections
- Autoimmunity and lymphoid malignancies
13
Q
Selective IgA Deficiency
A
- B-cell disorder
- Most common primary immunodeficiency
- Lack IgA
- Asymptomatic, but can have recurrent infections, allergies, autoimmune
- Can progress to Common Variable Immunodeficiency
14
Q
Agammaglobulinemia
A
- B-cell disorder
- Defect in Bruton tyrosine kinase causes pre-B-cell maturation arrest, so underproduce all Ig types
- Lack B-cells in peripheral blood
- X-linked (so boys>girls)
- Treat with Ig injections
15
Q
Common Variable Immunodeficiency
A
- B-cell disorder
- Occurs in ADULTS (unlike most are kids)
- Most common symptomatic primary immunodeficiency
- Normal #s of B-cells, but dysfunctional
- Recurrent infections starting in adulthood
- Treat with Ig injections
16
Q
Chronic Granulomatous Disease
A
- Phagocytic disorder
- X-linked (so boys>girls)
- Mutation in NADPH oxidase causes defect in superoxide production
- Usually manifest in childhood
- Recurrent dangerous infections with catalase-+ bacteria (s. aureus), fungi, tissue granuloma formation (hence chronic GRANULOMATOUS)
- pulmonary, cutaneous, lymphatic, hepatic infections common
17
Q
Chediak-Higashi Syndrome
A
- Phagocytic disorder
- rare autosomal recessive
- Defect in protein transporting stuff to lysosomes
- Abnormal granules in neutrophils, melanocytes, hair, Schwann cells, CNS
- Recurrent childhood cutaneous and pulmonary infections
- Other symptoms: Albinism, retardation, neuropathy, lymphoproliferative syndrome (if survive to adulthood)
18
Q
Complement Deficiencies
A
- 1-4: infection prone, autoimmune risk
- 5-9: Neisseria infections
