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

[Sullivan, Kathleen]

Low T cells are relatively common in complex congenital heart defects even without a thymectomy.  We have seen one case turnaround after a heart transplant so we think it has to do with lymph recirculation.

On Apr 5, 2012, at 6:03 PM, Rafael Firszt wrote:


> 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 <image001.gif>

> 30 to 66

> %

> * 19 L

> * 26 L

> Absolute CD4 <image001.gif>

> 410 to 1800

> /uL

> * 131 L

> * 144 L

> % CD45RO <image001.gif>

> 12 to 37

> %

> * 15

> * 26

> Absolute CD45RO <image001.gif>

> 200 to 980

> /uL

> * 110 L

> * 158 L

> % CD45RA <image001.gif>

> 6 to 37

> %

> * 2 L

> * 2 L

> Absolute CD45RA <image001.gif>

> 130 to 1100

> /uL

> * 15 L

> * 12 L

> CD4:CD8 Ratio <image001.gif>

> 0.70 to 4.60

> ratio

> * 1.00

> * 0.96

> % CD8 <image001.gif>

> 13 to 47

> %

> * 19

> * 27

> Absolute CD8 <image001.gif>

> 180 to 1200

> /uL

> * 131 L

> * 153 L

> % CD3 <image001.gif>

> 60 to 87

> %

> * 38 L

> * 53 L

> Absolute CD3 <image001.gif>

> 520 to 2400

> /uL

> * 265 L

> * 296 L

> % CD19 <image001.gif>

> 7 to 23

> %

> * 45 H

> * 34 H

> Absolute CD19 <image001.gif>

> 99 to 570

> /uL

> * 317

> * 192

> % Natural Killer Cells <image001.gif>

> 4 to 29

> %

> * 16

> * 12

> Absolute Natural Killer Cells <image001.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 <image001.gif>

> 44-441

> mg/dL

> * 54

> * 60

> * 43 L

> IGG <image001.gif>

> 700-1600

> mg/dL

> * 155 L

> * 221 L

> * 129 L

> IGM <image001.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 <image001.gif>

> ug/mL

> * 1.34

> Pneumo. Serotype 3, IgG <image001.gif>

> ug/mL

> * 0.76

> Pneumo. Serotype 4, IgG <image001.gif>

> ug/mL

> * 0.66

> Pneumo. Serotype 5, IgG <image001.gif>

> ug/mL

> * 2.34

> Pneumo. Serotype 6B, IgG <image001.gif>

> ug/mL

> * 2.28

> Pneumo. Serotype 7F, IgG <image001.gif>

> ug/mL

> * 1.94

> Pneumo. Serotype 8, IgG <image001.gif>

> ug/mL

> * 1.73

> Pneumo. Serotype 9N, IgG <image001.gif>

> ug/mL

> * 0.39

> Pneumo. Serotype 9V, IgG <image001.gif>

> ug/mL

> * 1.11

> Pneumo. Serotype 12F, IgG <image001.gif>

> ug/mL

> * 0.19

> Pneumo. Serotype 14, IgG <image001.gif>

> ug/mL

> * 3.25

> Pneumo. Serotype 18C, IgG <image001.gif>

> ug/mL

> * 2.29

> Pneumo. Serotype 19F, IgG <image001.gif>

> ug/mL

> * 3.19

> Pneumo. Serotype 23F, IgG <image001.gif>

> ug/mL

> * 1.38

>  

> Diphtheria/Tetanus Antibody Titer

>  

> Last Ref. Range

> Units

> 04/04/12

> 12:40

> Test Status

> Final

> Diphtheria Ab, IgG <image001.gif>

> IU/mL

> * 1.4

> Tetanus Ab, IgG <image001.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


Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


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