[PAGID] Candidiasis and cellular dysfunction in a previously healthy adult

[Bodo Grimbacher]

Dear Richard,
I could test for a new genetic defect we found causing CMC with late 
onset in some individuals (unpublished).
Alternatively: how about anti-IFN antibodies or something like that, as 
seen in APECED?
Yours, Bodo Grimbacher
UCL, London

> I'd very much appreciate comments and suggestions for further work up 

> of this patient.

> Richard Wasserman

> Dallas

>

> The case:

> I saw a 32 yo woman with candida esophagitis. She had been completely 

> well until several months after the birth of her second child when she 

> developed malaise, fatigue and non-specific GI symptoms of bloating, 

> abdominal pain and intermittent diarrhea. She has lost more than 10% 

> of her body weight and is significantly below her pre-pregnant weight. 

> She has been on a high calorie diet and continues to loose weight. She 

> has had an extensive GI evaluation including EGD, colonoscopy and 

> capsule study; all normal. There have been no other infections (no 

> significant infection history ever), no signs nor symptoms of collagen 

> vascular disease. Her studies of cellular immunity are:

>

>

> Abs.CD19+ Lymphs          245 /uL                    12-645

> Absolute CD 3            1433 /uL                    622-2402

> Absolute CD 4 Helper      1053 /uL                    359-1519

> Abs. CD 8 Suppressor      326 /uL                    109-897

> % CD19+ Lymphs            13.6 %                      3.3-25.4

> % CD 3 Pos. Lymph.        79.6 %                      57.5-86.2  % CD 

> 4 Pos. Lymph.        58.5 %                      30.8-58.5

> % CD 8 Pos. Lymph.        18.1 %                      12.0-35.5

> CD4/CD8 Ratio            3.23                        0.92-3.72  

> WBC                      5.4 x10E3/uL                4.0-10.5

> Hemoglobin                13.0 g/dL                  11.5-15.0

> Neutrophils              56 %                        40-74           

> Lymphs                    34 %                        14-46  

> Monocytes                8 %                        4-13  

> Eos                      1 %                        0-7  

> Basos                    1 %                        0-3            

> Tests: (3) Lymphocyte Activation (826963)

> Unstimulated CD4          12 ng/mL

> PHA - CD4 Activation [L]  119 ng/mL           >225             Normal 

> activation of CD4 cells is indicated if the

> patient's responses reach the threshold or

> reference value shown.  The cellular immune

> response to PHA stimulation can be categorized as

> follows:

> ng/mL of ATP        Interpretation

> <226                  Low Immune Cell Response

> 226-524            Moderate Immune Cell Response

> >524                 Strong or High Level of Immune Cell Response

>

> Tests: (4) Tetanus toxoid-CD4 Activation (827140)

> Tetanus toxoid-CD4 Act.*

>                     [L]  10 ng/mL                    >23  This test 

> was developed and its performance

>

> characteristics determined by IBT Reference Lab.

> It has not been cleared or approved by the FDA.

> **Effective October 29, 2008 this panel will be**

> non orderable. For replacement number, please

> contact your local LabCorp Representative.

> placement number, please contact

> your local LabCorp Representative.

>

> Tests: (5) Concanavalin A(CON A)-CD4 Act. (827144)

> Concanaval A (Con-A)-CD4 Act.*

>                           189 ng/mL                  >136

> This test was developed and its performance

> characteristics determined by IBT Reference Lab.

> It has not been cleared or approved by the FDA.

>

> Tests: (6) Varicella-Zoster-CD4 Act. (827138)

> Varicella-Zoster-CD4 Act.*

>                     [L]  14 ng/mL                    >22

> This test was developed and its performance

> characteristics determined by IBT Reference Lab.

> It has not been cleared or approved by the FDA.

>

> Tests: (7) Candida albicans-CD4 Act. (827134)

> Candida albicans-CD4 Act.*

>                     [L]  13 ng/mL                    >25  This test 

> was developed and its performance

> characteristics determined by IBT Reference Lab.

> It has not been cleared or approved by the FDA.

>

> Tests: (8) ANA w/Reflex if Positive (164863)

> ! Antinuclear Antibodies Direct

>                           38 AU/mL                    0-99             

>                               Negative        <100

>                               Equivocal  100 - 120

>                               Positive        >120

>

>

>

> Tests: (9) Sedimentation Rate-Westergren (005215)

> Sedimentation Rate-Westergren    5 mm/hr       0-20          Tests: 

> (10) C-Reactive Protein, Quant (006627)

> C-Reactive Protein, Quant    2.7 mg/L           0.0-4.9