[CIS PIDD] [cis-pidd] PFAPA and low memory B cells
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Sun Jul 17 03:03:37 EDT 2016
Hi Richard:
Despite the fact that she meets the criteria for PFAPA, her age and the abnormalities in her T and B cell flow argue that this is something else. Any chance this could be HSV-related? I have seen a patient in the past who was having major cervical lymphadenopathy and fevers every 3-4 weeks (had even had a lymph node evaluation by our H/O docs) who did very well on prophylactic acyclovir. This could also explain the expanded CD8 T cell subset. The thyroid disease could suggest activated STAT1 although I cannot find any reports of actual thyroid carcinoma in these patients – they certainly can have problems with HSV and aphthous stomatitis and some have selective IgA deficiency.
Prescott
T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel 205-996-9582
Fax 205-975-7080
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Saturday, July 16, 2016 4:26 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] PFAPA and low memory B cells
She was treated with prednisone 80mg one dose before I saw her without benefit. I plan to try it again. Her episodes are too frequent (more than one per month) to use steroids routinely. I have been thinking about canakinumab but am concerned in the context of an apparent antibody production abnormality. I think that the best next step is probably tonsillectomy but I am not optimistic.
Thank you for the comments and suggestions.
Richard
On Sat, Jul 16, 2016 at 9:00 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
i agree with prednisona 1 mg/kg on the first day, single dose, on demand ( its abortive). if it requires corticosteroids often, try tonsilectomy. colchicine its not an option. In adults there are some reports of refractory pfapa treated with canaquinumab 150 mg SC 6/6 weeks.
2016-07-15 20:50 GMT-03:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:
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><mailto: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.gonzalez at salud.madrid.org><mailto:luisignacio.hdoc 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><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><mailto: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<tel:%28972%29%20566-7788>
Fax (972) 566-8837<tel:%28972%29%20566-8837>
Cell (214) 697-7211<tel:%28214%29%20697-7211>
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Médico do Serviço de Imunologia Clínica e Alergia Hospital Das Clinicas, Faculdade de Medicina da Universidade de São Paulo
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Allergy Partners of North Texas
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