Immunodeficiency Flashcards
Lack of antibody response cause what type of infections
Recurrent sinopulmonary and gut infections
Infections by polysaccaharide encapsulated pyogenic organisms
Infections predisposed to with lack of T cells
Infections with intracellular organisms -AIDS
Fungi -candida
Viruses
Listeria
Lack of neutrophils/monocytes cause which infections
High grade bacterial infections -Staph.aureus, G-ve
Fungi - Invasive aspergillus, systemic candidiasis
Lack of alternate pathway (properdin/Factor D)
Neisseria infections
Which infection is linked to iron deficiency
Candida
Assessment of antibody
Dynamic antibody response to vaccination
Assessment of complement deficiency
CH 50- measures Classical pathway
AH 50 -measures alternate pathway
CVID characteristics
Any age, often no family history
Presents with recurr sinopulmonary infections, gut,skin
T cell infections uncommon
Often associated with IgA deficiency
Neoplasia -400 x risk of lymphoma, gastric cancer
Autoimmune disorders in 20% (ITP,AIHA,PA)
Criteria for diagnosis of CVID
IgG low, Ig A or M also decreased
B cell count close to normal. Normal T and NK cells
Impaired vaccination response
Treatment- IV Ig monthly, antibiotics for infections
X linked agammaglobulinemia characteristics
Early onset(6 mon)
No lymphoid tissue
Mutation in Btk (signalling molecule essential for B cell development)
Family history in 50%
LRTI,GI infections, malabsorption,polyarthropathy
Diagnosis of Brutons
EPG -hypogamma IgG levels -typically undetectable B cell count - 0 B-cell precursors absent in marrow Btk expression by flow
Disease association in Ig A deficiency
Atopic disease, asthma
Cows’s milk allergy
Coeliac - can have false negative results as TTG absent
Anaphylaxis on transfusion of IgA containing blood prod
AI disease- RA,JRA,SLE, Sjogrens,ITP,Pernicious anemia
Which immunodeficiency can have anaphlaxis with blood transfusion
IgA deficiency
Due to Anti-IgA antibodies
Characteristics of hyper-IgM syndrome
Age of onset 1-2 yrs
X linked,CD40 ligand deficiency
Absent CD40-CD40L signal in Tcell-B cell collaboration->
failure of Bcell isotype switching and memory B cells
Recurrent bacterial infections
Respiratory -esp PCP
A/c or chronic diarrhoea-cryptosporidium
Neutropenia
Diagnosis of Hyper IgM
Markedly decreased IgA,IgG,IgE
Normal or increased IgM
Normal circulating B cells
Flow cytometry for surface CD40L
Rx-IV Ig, bactrim Px, G-CSF
Characteristic of chronic mucocutaneous candidiasis
Onset in childhood
Affect skin, nails, mucosae
Oesophagial and pulmonary
Diagnosis - Lack of Th17 cells
Characteristics of SCID
Pediatric
Failure to thrive,chronic diarrhoea
Recurrent opportunistic infections(PJP)
Diagnosis- Lack of component essential for Tcell function
ie,Gamma common chain,ADA
IL-7 -most affected- needed for T and NK cells
Chronic granulomatous disease characteristics
Typically pediatric
Deficiency of 1 of 4 subunits of NADPH oxidase
Respiratory burst of neutrophils necessary for killing of intracellular organisms
Recurrent infections of skin,lungs- CoNS
Recurrent bronchopneumonia,Crohns like syndrome
Diagnosis -NBT
