[CIS PIDD] [cis-pidd] Hypogammaglobinemia

[CIS-PIDD]

Wondering if you have checked stool for norovirus by pcr?

MichaelGottlieb
Los Angeles

On Mon, Mar 6, 2017 at 8:56 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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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