[CIS PIDD] [cis-pidd] Markedly reduced T cells with normal immunoglobulins

Mon Sep 11 22:08:50 EDT 2017

Thanks everyone for your valuable inputs.

The antibodies used were anti - CD3.
For NK cells, only anti - CD56 was used. (CD16 was not used).

The test was repeated twice and the results were similar. CD45 positive -
lymphocytes were first gated and then the rest of the gating was carried
out.

CD4 count was 261,  and CD8 was 946.

I would try looking at recent thymic emigrants, naive and memory T cells.

Dear Dr Fabian
I shall share the raw files in next 24 hours.

As the child continues to remain sick, should I consider giving a dose of
IVIG?

Will keep the group posted on the developments....

Regards
Sagar

*Dr.Sagar Bhattad*
MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI, CHD)
*Giannina Gaslini Institute, Italy (Observership in Rheumatology)*
Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI Hospitals,
Bengaluru
*http://www.pediatricimmunologist.in/
<http://www.pediatricimmunologist.in/>*

*Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar,
Bengaluru -560 092, Karnataka*
* (**Book appointment - 08043420100)*

On Mon, Sep 11, 2017 at 7:57 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

>
>
> Dr. Bhattad:
>
>
>
> I agree with the responses of Drs. Sullivan and Hauck.  I’ve certainly
> seen T cell counts drop to the floor in the setting of severe infection
> (and, even not-so-severe infection).
>
>
>
> The flow results are weird.  But, I think this would be a case of the
> devil-in-the-details.  I suggest harassing the person who did the flow
> cytometry.  Do the plots look good, or, was the staining all suboptimal?
> (if people were skimping on the antibody, this would be why a population
> would not gate properly).  More importantly – what antibody did they use to
> identify “T cells”?  If they used anti-TCRalpha/beta, or anti-CD4 +
> anti-CD8 with the same color (rather than anti-CD3), then this probably
> gives the answer to the case (the so-called “leaky SCID with gamma/delta T
> cells”).  For “NK cells”, did they use both anti-CD16 and -CD56, or just
> one?
>
>
>
> If all the stainings were done right, and you do have a bonafide
> non-CD3/4/8/19/16/56 lymphocyte (CD45+) population, you would get some
> innate immunologists rather excited.
>
>
>
> Good luck.
>
>
>
>     - Karl
>
>
>
> *From:* cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] *On
> Behalf Of *CIS-PIDD
> *Sent:* Monday, September 11, 2017 4:16 AM
> *To:* CIS-PIDD
> *Subject:* AW: [cis-pidd] Markedly reduced T cells with normal
> immunoglobulins
>
>
>
> Dear Sagar,
>
>
>
> I am not aware of a malnutrion condition that selectively reduces T cells
> but leaves intact B and NK cells. However, I am aware of several conditions
> that because of a T cell deficiency lead to a wasting disease.
>
> Having said that and looking at the reduced T cell counts and the "missing
> lymphocyte population" to reach 100%, I would like to ask you the details
> of your gating strategy first.
>
> Hypergammaglobulinemia does not exclude a T cell deficiency.
>
> I would first consider TCRalpha or CD3-complex  deficiency, but almost all
> clasical (S)CID genes could be the cause.
>
> You could do flow cytometry again and closesly look at the raw data (MFI):
>
> - CD4 and CD8 T cells
>
> - Recent thymic emigrants
>
> - Naive and memory T cells
>
> - alpha/beta and gamma/delta T cell
>
> - TCR Vbeta repertoire
>
> - T cell proliferation
>
>
>
> Just send me a private message if you want us to do the flow cytometr for
> you.
>
>
>
> Best, Fabian
>
>
>
> *PD Dr. med. Dr. sci. nat. Fabian Hauck*
>
>
>
> Oberarzt / Leiter Immundefektambulanz und Immundiagnostisches Labor
> Kinder- und Jugendarzt / Kinderhämatologe und -onkologe / Fachimmunologe
> (DGfI)
>
>
> *Dr. von Haunersches Kinderspital*
> Klinikum der Universität München
> Lindwurmstr. 4, 80337 München
> <https://maps.google.com/?q=Lindwurmstr.+4,+80337+M%C3%BCnchen&entry=gmail&source=g>
>
>
>
> Tel.: (089) 4400-53931
>
> Fax: (089) 4400-53964
> E-Mail: *fabian.hauck at med.uni-muenchen.de
> <fabian.hauck at med.uni-muenchen.de>*
>
>
>
>
>
> Das Klinikum der Universität München ist eine Anstalt des öffentlichen
> Rechts (AöR)
>
> The Klinikum der Universität München is an Institution under Public Law
>
>
>
>
>
>
> ------------------------------
>
> *Von:* cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> im Auftrag
> von CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> *Gesendet:* Montag, 11. September 2017 09:10
> *An:* CIS-PIDD
> *Betreff:* [cis-pidd] Markedly reduced T cells with normal immunoglobulins
>
>
>
> Dear all
>
>
>
> I need your inputs in the following case......
>
>
>
>
>
> 17 months old boy, unwell for the past 4 months. Born to consanguineously
> married couple. Other siblings healthy.
>
> Repeated episodes of oral thrush for the past 4 months. Some improvement
> with application of oral clotrimazole. Poor intake of food and loss of
> weight (10 kg to 9 kg). Admitted with pneumonia for the past 15 days.
>
>
> On examination
>
> Oral thrush; features of severe malnutrition (Kwashiorkor)., pedal edema;
> Chest - bilateral crepitations
>
>
>
> Investigations
>
> Anemia
>
> No neutropenia
>
> Lymphocyte counts 3700
>
>
>
> Low albumin
>
> Other liver functions, renal functions - normal
>
>
>
> HIV - Non reactive
>
>
>
> IgG 1640 (High)
>
> IgA   523 (High)
>
> IgM  110
>
> IgE  < 1.5
>
>
>
> NBT - normal
>
> DHR - normal
>
>
>
> T - 7%
>
> B - 24%
>
> NK - 5 %
>
>
>
> Absolute counts
>
> T - 332
>
> B - 1137
>
> NK - 227
>
>
>
>
>
>
>
> Repeat testing after 2 days
>
> T - 11%
>
> B - 31%
>
> NK - 7.7 %
>
>
>
> Absolute counts
>
> T - 430
>
> B - 1172
>
> NK - 292
>
>
>
>
>
> T cells are markedly reduced (repeated twice, 2 days apart). What are the
> conditions in which T cells are markedly reduced in presence of normal/high
> IgG?
>
>
>
> Is it a variant of SCID?  Leaky SCID?
>
> (The first year of life was uneventful and this, to my knowledge is very
> odd for SCID)
>
>
>
> Does severe malnutrition cause a selective reduction in T cells?
>
>
>
> How should I evaluate this child further? Any role of IVIG in this setting?
>
>
>
> The sum of % of T, B and NK is around 36% on first occasion and 49 % on
> the repeat sample ...... What could be the reasons for this? The lab expert
> feels it could be due to debris (but in this case there was no debris and
> she thus had no clue as to why the sum was not 100%)
>
>
>
> Kindly provide your valuable inputs
>
>
>
>
>
> Regards
> Sagar
>
>
>
>
>
>
>
> *Dr.Sagar Bhattad*
>
> MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI, CHD)
>
> *Giannina Gaslini Institute, Italy (Observership in Rheumatology)*
>
> Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI
> Hospitals, Bengaluru
>
> *http://www.pediatricimmunologist.in/
> <https://urldefense.proofpoint.com/v2/url?u=http-3A__www.pediatricimmunologist.in_&d=DwMGaQ&c=ZcS_IThVDLRgSnibLQVJ9vwqRPpc3RkFqvJL1VfvJu0&r=SS5WX_zJKFGQt9gnWxXM2zj3mGuaCrOGfogIF4rsPm06T2ggGCIyqTpu8I8GvD7y&m=-6mZnv6iHcpGF419KfenN4koLYGcFnQpGMDuVPjlLUs&s=pBu5froWgSNWtRYqyUAnoVrK745fYRQ7X4gU2kZw2V8&e=>*
>
> *Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar,
> Bengaluru -560 092, Karnataka*
>
> * (**Book appointment - 08043420100**)*
>
>
>
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