[CIS PIDD] [cis-pidd] Hypogammaglobinemia

Thu Mar 23 09:33:02 EDT 2017

>
> Good Morning,
>

I sent this case before. But I want to send it again becasue since my last
submission she has been admitted 2 more times for severe diarrhea,
dehydration and some vomitting. She has not been scoped yet because she
would not let GI during her last admission and there were plans to do
outpatient but now she is admitted again.  They think she also has another
UTI which she keeps getting, and I referred the patient to urology to see
if its a bladder emptying issues but the patient hasn't followed through.
She was just infused with IVIG  less than a week ago and her level
preinfusion was 1120.  I asked them to send Norovirus and enterovirus in
the stool.  I also had rechecked her Cell Markers last week, and her Ts and
NKs are normal, but her B cell (CD19) is low which it never has been
before. It is 88 (141-448) . HIV neg.  Haven't been able to do oxidative
burst and Mitrogen to complete the work up.
IgM is still normal but dropping each year. Currently is 107, prior to that
was 127, 145, 147. IgA has been normal, except it was randomly checked 3
weeks ago and dropped to a low level of 71, but rechecked last week its
back to normal  I was working on getting her into genetics to test for CVID
but that hasn't happened yet and if negative pursue whole exome
sequencing.

Anyone have any input as to what could be the cause of her recurrent
diarrhea/illness.  I don't have her on any prophylaxis. I was considering
Bactrim but I don't know how much that will help with her diarrhea, but
maybe with the UTIs. She did have one episode of CDiff but all the episodes
afterwards CDiff is negative (and her CDIFF was difficult to treat, two
rounds of flagyl and one of vanco).

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 (However
she disclosed to me she drinks 2 beers a night), 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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