[CIS PIDD] [EXTERNAL] [cis-pidd] Hypogammaglobinemia

Thu Mar 23 16:54:31 EDT 2017

Thank you!!!

On Thu, Mar 23, 2017 at 4:52 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> We have used the Cincinnati Children’ s lab.
>
> Charlotte Cunningham-Rundles
>
>
> On Mar 23, 2017, at 4:46 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> NIH and us :-)
> ****************************************
> 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
> <https://urldefense.proofpoint.com/v2/url?u=http-3A__www.uniklinik-2Dfreiburg.de_cci&d=DwQF-g&c=shNJtf5dKgNcPZ6Yh64b-A&r=9wDVY2VgaedQ87MgHg121rcA_a89G8zsGBKnHHiX-bgDuWGOy2QBR3IOxzZd9meZ&m=yW4Uai8Sl5d4zxTSd7U6hEqzHSD2E_VlczOidYo06t4&s=ZudL4R1dJNv-ZyAHYC1A8K8uUQQzDO7v_-VeUprjZx8&e=>
>
> Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Datum: Thursday 23 March 2017 21:41
> An: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Betreff: Re: [cis-pidd] Hypogammaglobinemia
>
> 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
>> <https://urldefense.proofpoint.com/v2/url?u=http-3A__www.uniklinik-2Dfreiburg.de_cci&d=DwMF-g&c=shNJtf5dKgNcPZ6Yh64b-A&r=9wDVY2VgaedQ87MgHg121rcA_a89G8zsGBKnHHiX-bgDuWGOy2QBR3IOxzZd9meZ&m=yW4Uai8Sl5d4zxTSd7U6hEqzHSD2E_VlczOidYo06t4&s=ZudL4R1dJNv-ZyAHYC1A8K8uUQQzDO7v_-VeUprjZx8&e=>
>>
>> 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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> Charlotte Cunningham-Rundles MD PhD
> David S Gottesman Professor
> Department of Medicine
> 1425 Madison Avenue
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> Charlotte.Cunningham-Rundles at mssm.edu
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