[CIS PIDD] [cis-pidd] PFAPA, IgA Deficiency, no EBNA
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Tue Oct 18 10:41:54 EDT 2016
I am caring for a 19 year old woman with a long history of recurrent
sinusitis and respiratory tract infection now with a one year history of
debilitating classic episodes of Fever (39.5), cervical Adenopathy,
Pharyngitis and Aphthous stomatitis lasting five days and requiring a few
more days to recover occurring at least once a month. These episodes are
poorly responsive to prednisone (40mg once or twice a day). There is an
incidental new diagnosis of thyroid cancer.
It appears that she is not able to produce EBNA antibody (first IgG-VCA
positive > 1 year ago). Lab studies are below. I think she is not just a
Selective IgA deficiency.
There is a tentative plan for tonsillectomy. Are there alternate
suggestions for management of the PFAPA? If the tonsils are removed, what
immunohistologic studies would you get?
Thanks,
Richard Wasserman
*T- and B-Lymphocyte/Nat Killer (505370)*
Abs.CD19+ Lymphs 84 /uL 12-645
% CD19+ Lymphs [L] 2.8 % 3.3-25.4
Absolute CD 3 [H] 2553 /uL 622-2402
% CD 3 Pos. Lymph. 85.1 % 57.5-86.2
Absolute CD 4 Helper 834 /uL 359-1519
% CD 4 Pos. Lymph. [L] 27.8 % 30.8-58.5
Abs. CD 8 Suppressor [H] 1470 /uL 109-897
% CD 8 Pos. Lymph. [H] 49.0 % 12.0-35.5
CD4/CD8 Ratio [L] 0.57 0.92-3.72
Ab NK (CD56/16) 327 /uL 24-406
% NK (CD56/16) 10.9 % 1.4-19.4
*B-Cell Memory and Naive Panel (818314)*
! B-cells % CD19 [L] 3 % 5-26
! B-cells Absolute CD19
82 cells/uL 58-558
! Naive B-cell %CD19+/CD27-/IgD+
60 % 29-93
! Naive BCL Abs CD19+/CD27-/IgD+
49 cells/uL 22-423
! Non-switched Memory %
7 % 2-25
! Non-switch Abs 5 cells/uL 4-66
! Class-switched Memory %
[L] <1 % 3-23
! Class-switched Abs [L] 1 cells/uL 4-62
! IgM Only Memory % [H] 15.6 % .3-6.0
! IgM Only Memory Abs 12.8 cells/uL .6-16.4
! Total Memory B-cell%CD19/CD27+
23 % 7-48
! Tot Mem BCL Absol CD19+/CD27+
19 cells/uL 13-148
WBC 6.7 x10E3/uL 3.4-10.8
RBC 4.12 x10E6/uL 3.77-5.28
Hemoglobin 12.8 g/dL 11.1-15.9
Hematocrit 37.4 % 34.0-46.6
MCV 91 fL 79-97
MCH 31.1 pg 26.6-33.0
MCHC 34.2 g/dL 31.5-35.7
RDW 13.1 % 12.3-15.4
Platelets 247 x10E3/uL 150-379
Neutrophils 39 %
Lymphs 44 %
Monocytes 14 %
Eos 2 %
Basos 1 %
Neutrophils (Absolute)
2.6 x10E3/uL 1.4-7.0
Lymphs (Absolute) 3.0 x10E3/uL 0.7-3.1
Monocytes(Absolute) 0.9 x10E3/uL 0.1-0.9
Eos (Absolute) 0.1 x10E3/uL 0.0-0.4
Baso (Absolute) 0.1 x10E3/uL 0.0-0.2
Immature Granulocytes
0 %
Immature Grans (Abs) 0.0 x10E3/uL 0.0-0.1
*EBV* Acute Infection Antibodies (216655)
EBV Ab VCA, IgM <36.0 U/mL 0.0-35.9
Negative <36.0
Equivocal 36.0 - 43.9
Positive >43.9
EBV Early Antigen Ab, IgG
<9.0 U/mL 0.0-8.9
Negative < 9.0
Equivocal 9.0 - 10.9
Positive >10.9
EBV Ab VCA, IgG [H] 58.1 U/mL 0.0-17.9
Negative <18.0
Equivocal 18.0 - 21.9
Positive >21.9
EBV Nuclear Antigen Ab, IgG
<18.0 U/mL 0.0-17.9
Negative <18.0
Equivocal 18.0 - 21.9
Positive >21.9
*EBV PCR* Quant (Whole Blood) (139490)
! EBV PCR Quant(Whole Blood)
200 copies/mL Negative
The quantitative range of this assay is 100 to 1 million copies/mL.
This test was developed and its performance characteristics determined
by LabCorp. It has not been cleared or approved by the Food and Drug
Administration. The FDA has determined that such clearance or
approval is not necessary.
! log10 EBV DNA Qn PCR 2.301 log10copy/mL
*Immunoglobulin G*, Qn, Serum (001776)
Immunoglobulin G, Qn, Serum
1291 mg/dL 549-1584
Immunoglobulin A, Qn, Serum (001784)
Immunoglobulin A, Qn, Serum
[L] 9 mg/dL 87-352
**Verified by repeat analysis**
Immunoglobulin M, Qn, Serum (001792)
Immunoglobulin M, Qn, Serum
110 mg/dL 58-230
Immunoglobulin E, Total (002170)
Immunoglobulin E, Total
24 IU/mL 0-100
*Post vaccination titers:*
Pneumococcal Ab (23 Serotype) (812166)
Pneumo Ab Type 1* 11.4 ug/mL >1.3
Pneumo Ab Type 3* 9.8 ug/mL >1.3
Pneumo Ab Type 4* >15.8 ug/mL >1.3
Pneumo Ab Type 8* 7.6 ug/mL >1.3
Pneumo Ab Type 9 (9N)*
13.8 ug/mL >1.3
Pneumo Ab Type 12 (12F)*
11.9 ug/mL >1.3
Pneumo Ab Type 14* >31.0 ug/mL >1.3
! Pneumo Ab Type 17 (17F)*
16.4 ug/mL >1.3
Pneumo Ab Type 19 (19F)*
23.6 ug/mL >1.3
! Pneumo Ab Type 2* 4.8 ug/mL >1.3
! Pneumo Ab Type 20* 4.3 ug/mL >1.3
! Pneumo Ab Type 22 (22F)*
53.9 ug/mL >1.3
Pneumo Ab Type 23 (23F)*
2.0 ug/mL >1.3
Pneumo Ab Type 26 (6B)*
51.7 ug/mL >1.3
Pneumo Ab Type 34 (10A)*
>32.9 ug/mL >1.3
Pneumo Ab Type 43 (11A)*
12.8 ug/mL >1.3
Pneumo Ab Type 5* 6.5 ug/mL >1.3
Pneumo Ab Type 51 (7F)*
5.8 ug/mL >1.3
Pneumo Ab Type 54 (15B)*
>29.3 ug/mL >1.3
Pneumo Ab Type 56 (18C)*
5.2 ug/mL >1.3
Pneumo Ab Type 57 (19A)*
24.8 ug/mL >1.3
Pneumo Ab Type 68 (9V)*
18.8 ug/mL >1.3
Pneumo Ab Type 70 (33F)*
12.2 ug/mL >1.3
Haemophilus influenzae B IgG (138271)
Haemophilus influenzae B IgG
>9.00 ug/mL
Tetanus Antitoxoid IgG Ab (163691)
Tetanus Antitoxoid IgG Ab
>7.00 IU/mL <0.10
Interpretation:
Non-Protective <0.10
Protective >=0.10
Diphtheria Antitoxoid Ab (163709)
Diphtheria Antitoxoid Ab
1.09 IU/mL <0.10
Interpretation:
Non-Protective <0.10
Protective >=0.10
-
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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