[CIS PIDD] [cis-pidd] Hypogammaglobinemia

Mon Mar 6 12:33:39 EST 2017

No we have not. Will think of that next time. Currently her diarrhea has
resolved

On Mon, Mar 6, 2017 at 12:14 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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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