[CIS PIDD] [cis-pidd] Secondary amyloidosis and lack of memory T cells

[CIS-PIDD]

Dear all,

33yo male referred from our nephrology dept, where he was admitted with ESRD
and nephrotic syndrome, and was diagnosed with *secondary amyloidosis*.

Familial and personal history are negative for autoinflammatory or
autoimmune symptoms, however SAA is chronically elevated.
As a newborn he had *esophageal atresia*.

Prior medical history is hard to assess, however it seems he had early
onset, recurrent and *severe pulmonary infections* (bronchiectasias,
chronic bronchiolitis), serum IgG are now low-ish  but probably due to
protein loss, IgM and IgA are normal.

Leukocyte counts are normal, B lymphocytes phenotype is normal, T cell
however look like this: totally CD45RA+
[image: Inline image 1]
>From the literature I've found that CD45RA skewing can be seen in mutation
of the NFKB (IKBA, IKBKB) pathway and CBL signalasome (MALT1,
CARD11)... Anything
else that I should be aware of that might cause an immunological picture
like this?

He might  have had a growth defect (he is short). Skin is dry and hair is
scarse and thin, but it could be due to the end stage renal disease...
teeth are nails are normal.

How would you proceed?
I sent for:
- mitogen proliferation
- TLR response
- polysaccharide and vaccination responses

Thank you for your time!

Boaz Palterer, MD
Department of Clinical and Experimental Medicine
Unit of Allergology and Clinical Immunology
University of Florence
email: boaz.palterer at gmail.com
cell: +39 392 7169114

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