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

[Amos Etzioni]

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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