[CIS PIDD] [cis-pidd] PFAPA and low memory B cells
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Sat Jul 16 10:00:12 EDT 2016
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>:
> 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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--
*Dr Leonardo Oliveira Mendonça*
Médico do Serviço de Imunologia Clínica e Alergia Hospital Das Clinicas,
Faculdade de Medicina da Universidade de São Paulo
Unidade Convênios e Particulares/ Ambulatório de Doenças Autoinflamatórias
*Leonardo Oliveira Mendonça,MD*
Departament of Clinical Immunology and Allergy at Hospital das Clínicas,
School of Medicine - University of São Paulo
Private Healthcare Unit/ Autoinflammatory Disease Unit
email: *leonardo.mendonca at hc.fm.usp.br <leonardo.mendonca at hc.fm.usp.br>*
telefones/phone number: +55-11-26619571/ +55-11-26617825/ FAX: 011-2661 8173
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