CVID Flashcards
What criteria is used to characterise CVID??
Low IgG, and low IgM and/or IgA
What are classic infections seen with antibody deficiencies?
S aureus, s pneumoniae (strep), haemophilus influenza
Giardia, and enteroviruses.
When does CVID present vs other conditions with Ab deficiencies like XLA, SCID, CD40L and WAS?
Later onset, after 4 years of age.
What other things should you consider if presentation over 50 years?
Lymphoma and thymoma.
What are some of monogenic defects causing defective B cell development?
XLA, those involved in immune complex Iga/b (CD79) BLNK.
What are some of the monogenic mutatinos of CVID?
TACI and ICOS and CD19.
Most causes of CVID are polygenic.
What things are seen in CVIDs not seen in B cell defecs?
autoimmunity lymphoid malignancy and gastrointestinal complications (enteropathy)
Both T and B cells can be affected (to varying extents)
rare family history
variable phenotype
How would you treat CVID patients?
intial infections with antimicrobials and then with replacement Ig therapy.
Immunosuppressants if autoimmune.
Do CViD patients have <1% B cells?
What is phenotype assocaied with 90% of others?
around 10 per cent of CVID patients do.
reduced switched memory B clles (GC dysfunction) and elevated transitional B cells with CD21 low.
What is elevated transitional B cells associated with?
lymphoadenopathy
What is low CD21 populatinos assocaite with?
spenomegaly and granulomatous disease.
Phenotype of transitonal B cells?
And of low CD21 cells?
transiitonal B cells have CD38+ IgM+
CD21lo CD38lo
What sort of dysregulation of CD4 T cells can be seen in CVID?
lower total number
higher activatoin of T cells and Th1 responses
lower Tregs.
What sort of CD8 T cell dysregulatino is there in CD8+ T cells?
more activated phenotype.
