[CIS PIDD] [cis-pidd] Hypogammaglobulinemia and light chain deposition in kidneys

[Juthaporn COWAN]

Hello all,

Please advice what you think and what should be done for this case.

A 58 yo woman with history of mild mental retardation from fetal alcohol
syndrome. She was well up until 2009 when she was found to have
proteinuria. Kidney bx showed light chain deposition without evidence of
myeloma or even MGUS by bone marrow studies. She then had ITP +/- AIHA X 3
since 2009, responded to steroids and IVIG. She was also diagnosed with
bullous pemphigoid and pustular psoriasis in 2012 based on skin biopsy when
she presented with rash. Early this year, she began to have profuse
diarrhea lasted for several weeks and nothing found on work up in terms of
infection. Colonoscopy might have been done but result was not available.

We are seeing her for hypogammaglobulinemia of 1.1 g/L (Persistently low as
of this year). Her IgG level was 5.9 in 2009 when she was diagnosed with
light chain deposition in her kidneys and ITP. IgA and IgM are always in
normal range. Interestingly, she also has very low C4 level <0.001 g/L with
normal CH50. There is no feature of SLE.

The question is 'could this all be PID or SID?' Is her
hypogammaglobulinemia caused by protein loss? (serum albumin is noted to be
25.. not very low, proteinuria is about 1.5-2 g/day). Does she have
hematologic malignancy that is not picked up by BM biopsy?

No history of recurrent or unusual infection.

I am looking forward to hearing from you. Thank you.


Juthaporn Cowan
Ottawa, Canada

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