[CIS PIDD] [cis-pidd] Hypogammaglobinemia

[CIS-PIDD]

No we have not done that.  Is there a particular lab that does that test?

thank you

On Thu, Mar 23, 2017 at 3:40 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> CTLA4 tested?
>
> ****************************************
>
> Univ.-Prof. Dr. med. B. Grimbacher
>
>
>
> Scientific-Director
>
> CCI-Center for Chronic Immunodeficiency
>
> UNIVERSITÄTSKLINIKUM FREIBURG
>
> Tel.: 0761 270-77731  Fax: -77744
>
> Breisacherstraße 115, 79106 Freiburg
>
> bodo.grimbacher at uniklinik-freiburg.de
>
> www.uniklinik-freiburg.de/cci
>
> Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Datum: Thursday 23 March 2017 17:56
> An: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Betreff: Re: Re:[cis-pidd] Hypogammaglobinemia
>
> She used to have normal stool patterns until about November/December
> 2016.  But lately in between these episodes it ranges from loose to formed,
> and the only reason (I think) its formed is becasue she will take immodium
> to slow it down. But I suspect that without the immodium it would be more
> loose than formed.
>
> She had something similar to this back in 2009ish but a cause was never
> found.
>
> Thank you
>
> On Thu, Mar 23, 2017 at 9:40 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
>> Can you clarify – does she have normal stool pattern in between acute
>> illnesses or ongoing loose stools with discrete exacerbations?
>>
>>
>>
>> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
>> *Sent:* Thursday, March 23, 2017 9:33 AM
>> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
>> *Subject:* Re:[cis-pidd] Hypogammaglobinemia
>>
>>
>>
>> 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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