[CIS PIDD] [cis-pidd] Hypogammaglobinemia

Thu Mar 23 13:53:47 EDT 2017

Thank you! For the recurrent UTIs do you feel it is related to her immune
def? or likely an anatomy issue?  Any insight on how I should be treating
her otherwise?  Does she fit into any diagnosis you know of?  I was
thinking CVID more becasue her B cells are low and her IgA and IgM although
most of the time are normal have swayed into the low region before. Would
prophylaxis be warranted or is the E coli likely coming from the loose
stools? (But not always does she have a UTI but when does it is E Coli)

Thank you

On Thu, Mar 23, 2017 at 1:37 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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>
> 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]
>> *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>
>> wrote:
>>
>>> Have you tried Enteragam?
>>>
>>>
>>>
>>> Wellington S. Tichenor, M. D.
>>> 642 Park Avenue
>>> New York, New York 10065
>>> 212 517-6611 <(212)%20517-6611>
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>>>
>>> ------------------------------
>>> *From:* CIS-PIDD [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
>>> > 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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