[CIS PIDD] [cis-pidd] Hypogammaglobinemia

[CIS-PIDD]

Typically starting at 2 packets per day.  I have only used it on a couple of patients, but they did not have side effects.
________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, March 23, 2017 1:29 PM
To: CIS-PIDD
Subject: Re: Re:[cis-pidd] Hypogammaglobinemia

How is it dosed?  And they just take it everyday? Any side effects you have noticed with this medication?

I appreciate all the input!

On Thu, Mar 23, 2017 at 1:22 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
We have used it on a few patients with PI with significant help when no other cause was found.
________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Thursday, March 23, 2017 1:16 PM

To: CIS-PIDD
Subject: Re: Re:[cis-pidd] Hypogammaglobinemia

No we have not. I am not knowledgable about this medication and its indications.  Is that just for IBD?  Do you use it for CVID gut disease as well?  I don't know if she has CVID, (because her labs constantly change) but does it help?

Thank you!

On Thu, Mar 23, 2017 at 1:12 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Have you tried Enteragam?


Wellington S. Tichenor, M. D.
642 Park Avenue
New York, New York 10065
212 517-6611<tel:(212)%20517-6611>
wtichenor at sinuses.com<https://register.concentric.com/home/apps/mail/mbox_compose.cgi?pTo=wtichenor@sinuses.com>

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________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Thursday, March 23, 2017 12:56 PM
To: CIS-PIDD
Subject: 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<mailto: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<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Thursday, March 23, 2017 9:33 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto: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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