[CIS-PAGID] 18 year old with T cell lymphopenia

[dmvascon at usp.br]

Dear Rafael

We have seen some patients with complex congenital heart disease with  
very low T cell counts and even hypogammaglobulinemia (specifically  
IgG,like your patient) due to leakage of proteins (and cells) by  
serosal membranes.
They frequently present pleural effusions and sometimes peritoneal.

In a few cases of infants affected by these malformations presenting  
septic manifestations we replace IVIg (despite the fact that the IgG  
levels fall quickly) every week plus adequate high dose antibiotics.  
They usually improve after these measures allowing surgical approach  
to correct the heart defects.

All the best,

Dewton Vasconcelos
University of São Paulo

Citando Rafael Firszt <Rafael.Firszt at hsc.utah.edu>:


> Hi, I have what I think will be a relatively easy case but I wanted  

> to get some opinions on management of this patient:

>

> My patient is an 18-year-old female with a history of complex  

> congenital heart disease (HLH, TGA) and protein-losing enteropathy.  

> The

>

> She has been relatively well all her life except in November of this  

> year, she got admitted with spontaneous bacterial peritonitis (no  

> organism found). It improved with a relatively long course of  

> antibiotics.

>

> Previous infectious history included mild shingles at 12 years of  

> age and frequent URIs and possible sinus infections.  Never been  

> hospitalized for infections prior to this last admission.

>

> As part of her routine investigations, she was found to have a very  

> low lymphocyte count.

>

> She has had 2 lymphocyte enumerations: (the one in October is when  

> she was hospitalized) .  It shows pretty low T cell counts with  

> normalish B cells and Normal NK cells.  I am assuming this is likely  

> from her CHD surgery and removal of the thymus at birth.

>

>

> Last Ref. Range

>

> Units

>

> 04/04/12

> 12:40

>

> 10/24/11

> 20:15

>

> Test Status

>

> Final

>

> Final

>

> % CD4 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 30 to 66

>

> %

>

> * 19 L

>

> * 26 L

>

> Absolute CD4 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 410 to 1800

>

> /uL

>

> * 131 L

>

> * 144 L

>

> % CD45RO [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 12 to 37

>

> %

>

> * 15

>

> * 26

>

> Absolute CD45RO [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 200 to 980

>

> /uL

>

> * 110 L

>

> * 158 L

>

> % CD45RA [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 6 to 37

>

> %

>

> * 2 L

>

> * 2 L

>

> Absolute CD45RA [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 130 to 1100

>

> /uL

>

> * 15 L

>

> * 12 L

>

> CD4:CD8 Ratio [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 0.70 to 4.60

>

> ratio

>

> * 1.00

>

> * 0.96

>

> % CD8 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 13 to 47

>

> %

>

> * 19

>

> * 27

>

> Absolute CD8 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 180 to 1200

>

> /uL

>

> * 131 L

>

> * 153 L

>

> % CD3 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 60 to 87

>

> %

>

> * 38 L

>

> * 53 L

>

> Absolute CD3 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 520 to 2400

>

> /uL

>

> * 265 L

>

> * 296 L

>

> % CD19 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 7 to 23

>

> %

>

> * 45 H

>

> * 34 H

>

> Absolute CD19 [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 99 to 570

>

> /uL

>

> * 317

>

> * 192

>

> % Natural Killer Cells [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 4 to 29

>

> %

>

> * 16

>

> * 12

>

> Absolute Natural Killer Cells [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 68 to 570

>

> /uL

>

> * 110

>

> * 66 L

>

>

> In addition, her IgG has been very low which I am assuming is from  

> her pretty significant PLE.   She is on Entocort for this now but  

> was not on any medicines previously.

>

> Immunoglobulins (IgG, IgA, IgM), Serum    Show more...

>

>

>

> Last Ref. Range

>

> Units

>

> 04/04/12

> 12:40

>

> 10/14/11

> 21:15

>

> 10/03/11

> 12:35

>

> Test Status

>

> Final

>

> Final

>

> Final

>

> IGA [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 44-441

>

> mg/dL

>

> * 54

>

> * 60

>

> * 43 L

>

> IGG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 700-1600

>

> mg/dL

>

> * 155 L

>

> * 221 L

>

> * 129 L

>

> IGM [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> 48-226

>

> mg/dL

>

> * 51

>

> * 38 L

>

> * 40 L

>

>

> Her B cell Function is ok.  I did give her a pneumovax booster back  

> in October.

>

> Pneumococcal Abs, IgG

>

>

>

> Last Ref. Range

>

> Units

>

> 04/04/12

> 12:40

>

> Test Status

>

> Final

>

> Pneumo. Serotype 1, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 1.34

>

> Pneumo. Serotype 3, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 0.76

>

> Pneumo. Serotype 4, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 0.66

>

> Pneumo. Serotype 5, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 2.34

>

> Pneumo. Serotype 6B, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 2.28

>

> Pneumo. Serotype 7F, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 1.94

>

> Pneumo. Serotype 8, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 1.73

>

> Pneumo. Serotype 9N, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 0.39

>

> Pneumo. Serotype 9V, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 1.11

>

> Pneumo. Serotype 12F, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 0.19

>

> Pneumo. Serotype 14, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 3.25

>

> Pneumo. Serotype 18C, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 2.29

>

> Pneumo. Serotype 19F, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 3.19

>

> Pneumo. Serotype 23F, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> ug/mL

>

> * 1.38

>

>

> Diphtheria/Tetanus Antibody Titer

>

>

>

> Last Ref. Range

>

> Units

>

> 04/04/12

> 12:40

>

> Test Status

>

> Final

>

> Diphtheria Ab, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> IU/mL

>

> * 1.4

>

> Tetanus Ab, IgG [Description:  

> https://ihcweb-clin.co.ihc.com/ResultsReview/images/infoTrans.gif]

>

> IU/mL

>

> * 0.2

>

>

>

> However, her T cell function done back in October was low  

> particularly to PHA.  Her PWM was normal but since not specific for  

> T cells it's not that helpful.

>

>

> * Comments:

>

> 10/24/11.20:15 Result:

>

>

>

> Lymphocyte Mitogen Proliferation          See Note

>

>                Patient         Control 1       Control2

>                 CPM     SI*     CPM     SI*     CPM     SI*

> Media alone     1371    1       290     1       28968   1

>

> PHA 1:10        10431   8       939201  3239    1210707 42

> PHA 1:20        26791   20      1120279 3863    1103767 38

> PHA 1:50        38769   28      1041110 3590    975135  34

>

> CON A 1:20      4556    3       63665   220     450687  16

> CON A 1:40      13606   10      567123  1956    970666  34

> CON A 1:200     37760   28      589961  2034    711697  25

> CON A 1:400     23206   17      385869  1331    538510  19

>

> Media alone     1617    1       4203    1       1900    1

>

> PWM 1:10        99829   62      164237  39      228365  120

> PWM 1:20        168755  104     206033  49      237650  125

> PWM 1:40        260123  161     254229  60      217777  115

> PWM 1:200       354684  219     368383  88      318577  168

>

> Interpretation:

> Low Lymphocyte responses to PHA.

> Low Lymphocyte responses to Con A.

> Normal Lymphocyte responses to Pokeweed Mitogen.

>

>

>

>

>

> So I have several questions:

>

>

> 1)      Would you place this patient on Bactrim or any antibiotic  

> prophylaxis given low PHA, low CD4 counts?

>

> 2)      Given her good B cell responses, I am not going to put her  

> on IVIG but does that also make you more comfortable with the lower  

> T cell numbers and lower T cell mitogen stimulation?

>

> 3)      Would you order any additional tests?

>

> 4)      Are you in agreement, that the PLE is causing the low IgG  

> and the removal of thymus causing such persistently low T cell  

> counts and lower function?

>

> 5)      What happens if numbers get worse over time or she develops  

> a serious infection - would any of you ever consider transplantation?

>

> Thanks

>

> Rafael Firszt

>


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