[CIS-PAGID] 4 month old boy with high IgE and eosinophils...

[Bodo Grimbacher]

Dear all,
if you want to get fancy the lack of Th17 would point towards HIES
(STAT3 and Dock8) and the lack of Tregs would point towards IPEX.
Certainly both belong to the dd in this chap.
Yours,
Bodo Grimbacher
UCL, London, UK

> I believe measuring TREC can be a good test to find if the child has some kind of leaky SCID

> Amos etzioni

> ________________________________

> From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of 김예진 [yaejeankim at skku.edu]

> Sent: Friday, December 31, 2010 5:19 AM

> To: pagid at list.clinimmsoc.org

> Subject: [CIS-PAGID] 4 month old boy with high IgE and eosinophils...

>

>

> Dear All,

>

>

>

> I have a question about a 4 mo old boy with chronic diarrhea, eczema, hyper IgE and eosinophilia. This is a pt of my colleagues,  I already gave them my opinion but I also want to discuss the case with you all for further advice.

>

>

>

> This is a 4 mo old boy without any significant family history for PID. He developed generalized mild eczema at the age of 1 mo and developed diarrhea at 2 mo old. At 3 mo old, he also developed coughs. The child got hospitalzied.. they found hyper IgE and eosinophilia...rectal biopys revealed nothing.

>

> Diarrhea continued and respirtoary sx aggravated..BAL revealed PCP and CMV...

> They did immune work-up (see below of this email).   They suspect hyper IgE...

>

>

>

> Followings are my questions. Is this a typical presentation of hyper IgE? I would think Staph pneumonia more in a pt with hyper IgE.

> With PCP and CMV isolated and condition being improved with treatment, I would more suspect cell mediated immunity defect (fyi, HIV was negative).

>

> How about SCID (although lympho subset looks ok) or other cellular immunity defect? or how about IPEX? maybe hyper IgE should be ruled out anyway..

> Maybe hypereosinophilic syndrome such as eosinophilic gastroenteritis? even in this condition, CMV and PCP infection cannot explain..I don't think hyepreosinophilic syndrom pt have increased susceptibility to PCP or CMV.

>

>

>

> I would appreciate if you give any thoughts on this case.  Thank you all.

>

>

>

> YaeJean

>

>

>

>

> date    2010-11-08 18:15        2010-11-10 10:43        2010-11-15 14:12        2010-11-20 8:43 2010-11-29 9:49 2010-12-01 13:25        2010-12-02 11:22        2010-12-04 11:12        2010-12-08 9:39 2010-12-13 8:39 2010-12-16 10:08        2010-12-20 9:36 2010-12-23 9:22 2010-12-25 9:25

> CRP     0.82    3.02    　       0.44    1.47    0.98    2.16    0.9     0.74    1.93    　       0.74    　       2.27

> WBC     23.231  8.12    14.95   12.21   36.49   17.11   11.23   26.91   35.81   20.71   14.8    13.66   12.9    13.27

> Hb      9.4     5.5     9.8     8.3     8.6     6.3     9.1     10      9.8     10      8.8     9.6     9.4     8.8

> Platelet        551     260     107     124     227     290     317     338     450     264     296     323     220     179

> ESR     2       　       　       2       2       2       2       4       3       2       　       2       2       2

> Seg.neut.       42.7    38.9    33.5    30.1    28.8    38      46.1    55      29      32      70.7    36      49.5    46.8

> Lymphocyte      48.8    53.3    46.4    42.9    48.8    27.5    36.9    12      30.2    21.8    18.7    31.5    33.6    40.8

> Monocyte        7.9     5.7     6.2     13.4    6.9     9.2     11      8       9.4     8.8     6.2     7.2     7.9     5.8

> Eosinophil      0.4     2       13.4    13.3    15.1    24.8    5.4     25      31.2    37.3    4.3     25.1    8.8     6.5

> Basophil        0.2     0.1     0.5     0.3     0.4     0.5     0.6     　       0.2     0.1     0.1     0.2     0.2     0.1

>

>

>

> 　       2010-11-10 11:53        2010-11-20 9:52 2010-12-20 9:52

> IgG     671     759     528

> IgA     58      64      20

> IgM     64      89      87

>

>

>                 2010-12-20 9:26

>

> CD19(B cell)    248/㎕, 6%

> CD16/CD56(NK cell)      289/㎕, 7%

> CD3             3784/㎕, 88%

> CD4             2476/㎕, 57%

> CD8             1263/㎕, 29%

> Neutrophil DHR test

>

>

>

> normal

>

>

>

>

> 　

>         2010-11-08 18:14        2010-12-04 11:12        2010-12-08 9:39 2010-12-10 9:11 2010-12-13 8:39 2010-12-20 9:36

>

> Eos.count       130     306     11173   7345    7725    3429

>

> 　       2010-11-09 9:17 2010-11-20 10:01        2010-12-04 13:23        2010-12-10 10:24        2010-12-20 9:37

> IgE(Total)      1960    740     1930    1100    264

>

>

> --------------------------------------------------------------------------

>

> Yae-Jean Kim, MD

>

> Assistant Professor

>

> Division of Infectious Diseases

>

> Department of Pediatrics

>

> Sungkyunkwan University School of Medicine

>

> Samsung Medical Center

>

> 50 Irwon-dong Gangnam-gu

>

> Seoul, Korea

>

> Tel)  +82-2-3410-0987

>

> Fax) +82-2-3410-0043

>

>

>

>

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