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

[Church, Joseph]

Yae-Jean:


*         I think the S. aureus is secondary to the trach, neutrophil dysfunction of newborns and NICU environment.

*         The IgG level of 274 is within normal range for our lab (198-577), and should not predispose to Staph.

*         The eosinophilia may be a non-specific finding in sick neonates (Lawrence R et al Ann Allergy 1980 44;349-52).

I would follow, repeat B-cells and Ig's in a month.

I would avoid live virus vaccines, e.g. rotavirus for now.

Joe Church, MD
Children's Hospital Los Angeles




From: YaeJean Kim [mailto:yaejeankim at skku.edu]
Sent: Monday, November 26, 2012 8:58 PM
To: CIS-PIDD
Subject: [cis-pidd] a question about 6 week old boy with low B cells and heart anomaly

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<mailto:yaejeankim at skku.edu>


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