[CIS PIDD] [cis-pidd] Hypogammaglobinemia

[CIS-PIDD]

Good Morning,

I have inherited a patient from a previous immunologist and my group and I
are having trouble determining the cause of her immune deficiency and how
to manage her properly

She is 59 years old, with COPD, depression, recovering alcoholic,
hypothyroidism, smoker, has depression, OSA, T2DM, obese and has GERD and
an IVC filter and CHest port
She didn't have infection problems as a child, but starting in 1999 she
started having issues with frequent pneumonias and bronchitis as well as
thrush. Unfortunately EMR wasn't available in those days so I have many
holes in her documentation/history.
As per an immunologists note in 2009 she was having 1-2 PNA per year, 5-6
steroid bursts a year and sinusitis 3-4x a year.  No fam hx. Pt had a
miscarriage once.
An outside physician drew pneumococcal titers on her and administer
pneumovax and it was documented there was no significant change. But we do
not have those results available to us.
She has been intubated on multiple occassions and has had multipl episodes
of urosepsis. BCx + for EColi multiple times in 2012 and most recently in
2016. Also in 2012 her BCx was postiive for Bacillus species not B.
Anthracis
She has issues with vomitting and diarrhea many times and has been
hospitalized many times for it because of dehydration issues.  Giardia and
Crypto has been checked on 3 ocassions, always negative. Cdiff checked many
times and only positive once 12/2016 and was treated.
Also recently treated for HPylori

She was started on q3 weeklu IVIG in 2001 because of her immune workup
IgG was 375 7/2000 and in 2001 it was 480.  Initially her IgG subclasses
were normal but then the lastest in 2005 were IgG1, 3 and 4 were low (LABS
WILL BE AT THE END OF THE EMAIL)
One time in 2000 her IgA was slightly low for unknown reasons, but it has
been repeated many times with the most recent in 2016 and it has been
normal.
On one ocassion IgM was elevated at 200 but all the times after that
including in 2016 it was been in the normal range
Cell Markers in 2014 were CD4, CD8 and CD19 normal but CD16/56 was elevated
C3/C4 Nml
CT scan in 2016 does not note any bronchiectasis or emphysema
lAST pft fev1/fvc NORMAL, fev1 85% AND fef 25-75 reduced. Currently managed
by Pulmonary.
In 2000 her tetanus Ab was protective
2003 Sweat test was normal
Diptheria therapeutics
CT Abd/pelvis in 11/2016 was + for pyelonephritis during her most recent
episode of urosepsis.  colonic diverticulosis but no diverticulitis.  A few
periaortic retroperitoneal LNs one slightly more prominent compared to
prior examiniation, prabably reactive.

Over the years she continued to have repeat sinus issues and
hospitalizations so at some point her IVIG was changed to every 2 weeks and
that seemed to help a lot.
She currently receives 55gm of Flebo every 2 weeks with pretreatment. Her
IgG levels on this regimen had been 1021-1391 for the last 6 months.
However starting in November 2016 she had an episode of urosepsis with E
coli in her urine and blood, was in the ICU and intubated. Following that
she developed Cdiff (was treated with 2 rounds of Flagyl and Vancomycin in
order to resolve), following that she had an episode of bad vomitting and
diarrhea (determined to be viral) but needed to be admited to the hospital
of IV hydration for several days.  The recurrent dehydration and diarrhea
has been off and on and repeat Cdiffs after treatment have been negative.

Following that she had HPYlori and was being treated. While on that she
again had vomitting, diarrhea, came to the hospital severely dehydrated
with septic shock/hypovolemic shock was intubated on pressors (Cr 4, Na
114, Left shift on CBC) . Cultures in the urine, blood/port were all
negative (not sure if partially treated from HPYlori treatment)  and then
while in the hospital developed PNA and was also being treated for
sinusitis.  She missed her IVIG becasue of her acute illness and while
hospitalized she received her IVIG usual dose only 4 days after her
scheduled time. We checked her IgG prior to infusion and it had dropped to
842 and we also checked her pnuemococcal titers and only 15/23 were
>1.3 She is currently home and doing well. GI saw her while she was
admitted and recommended colonoscopy when she was stable but the patient
refused

We are having trouble controlling her recent spell of back to back
hospitalizations/illnesses and were curious on your thoughts as to what her
diagnosis could be and what we could be doing in addition to treat her
better.
We thought about prophylaxis, but got nervous becasue she needed so much
flagyl and vancomycin to treat her recent CDiff.
Would anyone increase her IVIG or frequency even more?

Her labs are attached in a Excel file.  I apologize if the formatting is
off and all over the place but thats the best that I could get it pulling
from the EMR

I appreciate everyone help and advice.
Pamella
Cleveland, OH

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