Immunodeficiencies Flashcards
adhesion and transmigration results from interactions between… except:
a) ICAMs
b) leukocyte integrins
c) sialyl lewis bodies CD15
d) selectins
c
Clinical Features
Early age
Hypoalbuminemia
Visceral abscesses
Recurrent sinopulmonary infection
Splenomegaly
Diarrhea
Malabsorption and growth delay
¿What systems are affected in recurrent infections in CGD?
Pneumonia
Liver abscess
Skin infection
Lymphadenitis
Osteomyelitis.
¿What type of pathogens has a pathological role in CGD?
a. Catalasa +
b. Catalasa -
Catalasa +
Main pathogens in CGD
S. aureus
B- cepacia
S. marcescens
Nocardia spp.
Asperguillus spp.
¿What is the pathogenesis of
rolling is a low affinity interaction mediated by… in the endothelial surface
a) P-selectin and e-selectin
b) CD15s
c) integrins CD18/CD11b
a
firm adhesion is a high affinity interaction between integrins …. in the leukocyte and …. on the endothelium
integrins CD18/CD11b, ICAMs
Bruton X-Linked Agammaglobulinemia:
Function loss of _______ → block in B cell maturation causing near total abscence of B cells in the periphery
a) CD40L
b) TACI
c) BTK
BTK
Consequence of BTK deficiency
a) Complete abscence of B Cells due to no proliferation, B-Cell development stops once they’re mature B Cells
b) Near complete abscence of B cells due to no proliferation, B-Cell development stops at Pre-B Cells.
Near complete abscence of B cells due to no proliferation, B-Cell development stops at Pre-B Cells.
When do recurrent infections in XL-A begin and why?
Men affected begin recurrent infections at 6 months old → because that’s the time it takes for the antibodies transfered from the mother to the baby to stop working
Characteristic clinical manifestations of XL-A EXCEPT:
a) Unusual sensitivity to infections by enterovirus
b) Diarrhea by G. lamblia
c) Recurrent upper and lower respiratory tract infections
d) Pulmonary Aspergillosis
d) Pulmonary Aspergillosis
Treatment of XL-A
Life-long IV infussions of immunoglobulin
Main mechanism of Hyper IgM syndrome
Block in the ability of B lymphocytes to switch from IgM to other isotopes
Diagnostic features of Hyper IgM EXCEPT:
a) Reduced IgA, IgE, IgG
b) Low numbers of B Cells
c) Normal and/or elevated IgM
Low numbers of B Cells
Hyper IgM presents NORMAL number of B cells
Deficiencies in type 1 and type 2 Hyper IgM:
a) CD40L (CD154) (1), AID (2)
b) CD18 (1), CD15 (2)
c) Common gamma chain (1) IL-1Ra (2)
d) RAG1 (1), RAG2 (2)
A) CD40 for type 1, AID for type 2
1 → deficiency in binding of B cells to CD40L
2 → deficiency in diferentiation because of AID deficiency
Cell count in Common Variable Immunodeficiency (CVID) / Hypogammaglobulina
NORMAL B Cells because of DEFECTIVE MATURATION
→ No plasma cells → Hypogammaglobulinemia
Diagnosis for CVID:
- Normal B Cells
- Low plasma cells
- Deficiency in more than 1 antibody
Cell count in yc (IL-2Ry) deficiency
T-, B+, NK-
Characteristics in yc (IL-2Ry) deficiency
Most frequent
Lack of T-Cells due to no maturaion, proliferation and activation
** X LINKED **
Fungal and microbial infections usully fatal
Cell count in IL-1Ra deficiency
T-, B+, NK+
Characteristics in IL-1Ra deficiency
Abnormal development of CD4 and CD8. NK cells are PRESERVED
Cell count in RAG1/2 deficiency (Also called Partial Lymphopenia)
T-, B-, NK+
Characteristics in RAG 1/2 deficiency
Deficiency in VDJ recombination in antibodies, receptors lose their ability to recognize antigens
Can cause Omenn Syndrome → rashes, eosinophilia, enlarged lymph nodes, diarrhea
Cell count in ADA deficiency
T-, B-, NK- (Also called Absolute Lymphopenia)
Characteristics of ADA deficiency
Mutations in Adenosine Deaminase causes accumulation of toxic purine nucleosides for B and T Cells
Clinical manifestations of SCIV
Oral trush (painless, can be scraped off)
