[CIS PIDD] [cis-pidd] a question about 6 week old boy with low B cells and heart anomaly

[YaeJean Kim]

 Dear all,

I have a question about 6 weeks old boy with very complicated heart disease
and slightly low B cells and mild eosniophilia.

This is a boy born at full-term with complicated heart disease and
tachycardia (PSVT/ atrial flutter).
The baby is in PICU on ventilator due to tachycardia and his tracheal
aspirate grew *S. aureus*. It was not sure whether he had bacterial
pneumonia although he had initially increased WBC count at 14.725x10^3/uL.

His CBC now is WBC 8.0 x10^3/uL, Hb 11.9g/dL, PLT 356K/uL with diff count
of S48, E12, L30, M8. (absolute eos counts 960/uL)
Initially, positive culture due to S. aureus from the tracheal aspirate and
mild eosinophilia at this age caught my eyes and I suspected hyper IgE
syndrome. (there is no family hx of PID, though).
So, we did the work-up and now the labs are as below..I see now he has
somewhat low B cells and low level of IgG, A, M (this could be within
normal range but lower end..).

IgG 274 mg/dL  (251-906)
IgA 5 mg/dL  (1.3-53)
IgM 9 mg/dL  (17-105)
IgE 8.8 IU/mL
C3, C4, CH50 normal

Lympho subset
CD3       2088/uL (87%),
CD4      1656/uL (69%),
CD8       384/uL (16%)
CD19      192/uL (*8%*)
CD16+56+CD3-  72/uL (3%)

 Pt with XLA usually present older than this age..and B cell is not that
low (it is above 1-2%)...The reason for eosinophilia is not known yet..
Do you think it is enough to suspect any forms of PID (such as hyepr IgE
or any form of hypogammaglobulinemia) at this stage?
I appreciate any feedback from you.

Thank you.

-- 
Yae-Jean Kim, MD, PhD
Associate Professor
Division of Infectious Diseases
Department of Pediatrics
Sungkyunkwan University School of Medicine
Samsung Medical Center
50 Irwon-dong Gangnam-gu
Seoul, Korea
Tel)  +82-2-3410-3539, 0987  Fax) +82-2-3410-0043
yaejeankim at skku.edu

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