[CIS PIDD] [cis-pidd] Patients with warts, CD4 lymphopenia and NK deficiency

[Jonathan Tam]

(not sure if this posted the first time I tried)

I have a patient that recently came in to see me as a referral from
dermatology for numerous and recalcitrant warts.  He is a 12 y/o M with a
history of static encephalopathy, microcephaly, seizure disorder, GERD,
g-tube dependence.  Born FT c-section with no complications.  Unremarkable
family history of non-consanguineous parents from Sri Lanka.  He was noted
to be small and a G-tube was placed at 9 mo.  He had his first seizure  in
2006 and has been stable on Keppra.  He is followed closely by GI; noted to
have rare intermittent diarrhea, but generally normal stools.   He is also
followed closely by genetics and was last seen 3/6/13.  To this point none
of the test have revealed a genetic defect (negative microarrray).

His infection history is pretty unremarkable.  In terms of infection, he
has had one episode of acute otitis media.  He has one possible
pneumonia/bronchitis.  He had a UTI and an "eye infection".  In total he
has required antibiotics 3-4 times lifetime.  However, starting 2 years ago
he started to have warts.  He has not responded to any of the therapies
including candida injections x3.

Any thoughts would be appreciated.

----------------------------------------

%CD3 (Total T Cell): 76.2

Absolute CD3 (Total T Cell): (L) 1000 Cells/uL

%CD3/CD4 (T Cell Helper): (L) 9.8 %

*Absolute CD4 (T Cell Helper): (L) 129 Cells/uL*

CD4+CD25+CD127dim 12%

*CD4+ CD45RA 10%*

%CD3/CD8 (T Cell Cytotoxic): (H) 57.3 %

Absolute CD8 (T Cell Cytotoxic): 753 Cells/uL

*%CD3+DR+: (H) 56 %*



*Mitogen blastogenesis to PHA, PWM and Con A all low.*

*Antigen blastogenesis to tetaus, candida, CMV, HSV, VZV and adeno all low.*


%CD19 (B Cell): 20.1 %

Absolute CD19 (B Cell): 264 Cells/uL

CD19+IgD+CD27- 48%

CD19+IgD+CD27+ 13%

CD19+IgD-CD27+ 26%



CD3-CD16,56+ % (Natural Killer): (L) 1.9 %

*Absolute NK ( Natural Killer): (L) 25 Cells/uL*

NK cytotoxicity (low, only sent once):

*                50:1        9%*

*                25:1        6%*

                12:1        5%

                6:1          3%



Tetanus AB Titer: 1.25 I.U./mL

H Flu (PRP) Titer: (L) 0.23 mcg/mL



WBC: 7.68 K/uL (Neut  70.1 % Lymph  17.1 % Mono  10.4 % Eos 1.7 % Baso 0.3
%)

HGB: 13.5 g/dL

MCV: 94.0 fL

PLTE: (H) 456 K/uL



Sed Rate:  14 mm/hr

CRP:  0.9 mg/dL



IgG 958 mg/dL

IgM 37 mg/dL

IgA  56 mg/dL

IgE  <2 KU/L



CH50 365 Unit



VCA IgG Index: 0.22 (05/05/14)

VCA IgG Interp: Negative (05/05/14)

VCA IgM Index: 0.11 (05/05/14)

VCA IgM Interp: Negative (05/05/14)

EBNA Index: 0.65 (05/05/14)

EBNA Interp: Negative (05/05/14)

EBV Specimen: Blood (05/05/14)

EBV PCR: No Epstein Barr Virus nucleic acid detected (05/05/1


---------------------------------------------------

Jonathan Tam, MD

Assistant Professor of Pediatrics

Division of Clinical Immunology & Allergy

Children’s Hospital Los Angeles

4650 Sunset Blvd, MS#75

Los Angeles, CA 90027

jstam at chla.usc.edu

Phone: 323.361.2501

Fax: 323.361.1191

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