[CIS PIDD] [cis-pidd] PFAPA and low memory B cells
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Sat Jul 16 01:04:41 EDT 2016
Dear Richard,
I have had some success with vitamin D supplements - there is some (though limited) data that low vit D contributes to intensity and frequency of febrile attacks.
Short course prednisone at onset usually successful indeed.
Best,
Isabelle Meyts
University Hospitals Leuven, Belgium
> Op 16-jul.-2016 om 01:51 heeft CIS-PIDD <cis-pidd at lists.clinimmsoc.org> het volgende geschreven:
>
> Have you tried prednisone? 1-2 days at the start of fever should stop it. I also agree with tonsillectomy since she has had this for some time
>
> Sent from my iPhone
>
> On Jul 15, 2016, at 4:40 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
>
> Dear Richard,
> from the pediatric perspective prior reported cure rates after adenotonsillectomy are even lower than experienced in clinical practice. PFAPA children usually have tonsil enlargement only within episodes. With long-lasting recurrent PFAPA episodes I would favour surgery in your patient.
> BTW I wonder if long-term antibody response will be maintained. However it seems to work fine in terms of antibody titers (despite low SmB cells)
> All the best,
> Nacho
>
> Luis I. Gonzalez-Granado. MD.
> Immunodeficiencies Unit.
> Hospital 12 de octubre.
> Research Institute Hospital 12 octubre (i+12)
> Av. Cordoba S/N. 28041. Madrid. Spain
> Tel. 0034606732959 / 0034913908569 / Fax 0034913908772<tel:0034934893039>
> luisignacio.gonzalez at salud.madrid.org<mailto:luisignacio.hdoc at salud.madrid.org>
> ORCID ID: orcid.org/0000-0001-6917-8980<http://orcid.org/0000-0001-6917-8980>
> Researcher ID: B-9257-2009
> ResearchGate:https://www.researchgate.net/profile/Luis_Gonzalez-Granado
> LinkedIn: https://es.linkedin.com/in/nachgonzalez
>
> 2016-07-15 21:56 GMT+02:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:
> Colleagues,
>
> I am seeing a 19 yo woman who has long carried the diagnosis of Selective IgA Deficiency. She has had multiple episodes of sinusitis and one pneumonia. For the past year, she has had textbook presentations of PFAPA with all four characteristics every 3-4 weeks. She recently had a thyroidectomy for thyroid cancer.
>
> Her laboratory studies are below. At this time she is being treated with prophylactic TMP/SMX. My questions are: What would you do for the PFAPA which is debilitating because of the 5-7 day duration of the episodes and their frequency (tonsils are visible but small). What additional laboratory studies may be of help?
>
> Thanks,
> Richard Wasserman
> Dallas
>
> Immunoglobulin A, Qn, Serum
>
> [L] 9 mg/dL 87-352
>
>
> Immunoglobulin G, Qn, Serum
>
> 1291 mg/dL 549-1584
>
> Immunoglobulin M, Qn, Serum
>
> 110 mg/dL 58-230
>
> 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
>
>
> Diphtheria Antitoxoid Ab (163709)
>
> Diphtheria Antitoxoid Ab
>
> 1.09 IU/mL <0.10
>
>
>
> 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
>
>
>
> ANA w/Reflex (164902)
>
> ANA Direct [A] Positive Negative
>
>
> Tests: (9) ENA+DNA/DS+Centro+Scl 70+Sj... (160033)
>
> Anti-DNA (DS) Ab Qn 2 IU/mL 0-9
>
> Negative <5
>
> Equivocal 5 - 9
>
> Positive >9
>
>
>
> RNP Antibodies 0.2 AI 0.0-0.9
>
> Smith Antibodies <0.2 AI 0.0-0.9
>
> Antiscleroderma-70 Antibodies
>
> [H] 1.2 AI 0.0-0.9
>
> Sjogren's Anti-SS-A <0.2 AI 0.0-0.9
>
> Sjogren's Anti-SS-B <0.2 AI 0.0-0.9
>
> Anti-Centromere B Antibodies
>
> <0.2 AI 0.0-0.9
>
> ANCA Panel (163873)
>
>
> Antimyeloperoxidase (MPO) Abs
>
> <9.0 U/mL 0.0-9.0
>
> Antiproteinase 3 (PR-3) Abs
>
> <3.5 U/mL 0.0-3.5
>
> Cytoplasmic (C-ANCA) <1:20 titer Neg:<1:20
>
> Perinuclear (P-ANCA) <1:20 titer Neg:<1:20
>
> Atypical pANCA <1:20 titer Neg:<1:20
>
> --
> 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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