[CIS PIDD] [cis-pidd] PFAPA and low memory B cells
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Sat Jul 16 17:25:42 EDT 2016
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>
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>:
>
>> 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
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--
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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