[CIS PIDD] [cis-pidd] LRBA and severe Thrombocytopenia with chronic CMV and EBV infection
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Thu Dec 29 11:57:13 EST 2016
Dear all,
My experience is based on 1 patient with LRBA deficiency and Evans syndrome: I agree with previous comments: steroids were only partially effective and short lasting. Rituxan followed by sirolimus has stabilized our patient.
Best regards,
Laia Alsina, MD, PhD
Adjunta de la Secció d'Immunoal.lèrgia; Unitat Funcional d'Immunologia, Hospital Sant Joan de Déu-Hospital Clínic.
Cap d'Estudis i Presidenta de la Comisió Local de Docència de l'Hospital Sant Joan de Déu, Barcelona.
http://orcid.org/0000-0002-3559-0018
El 29 dic 2016, a las 17:46, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> escribió:
I agree, rituxan is preferable and that BM exam is appropriate. Could also consider use of either eltrombopag or Romiplostim.
R
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Rob Sokolic, MD
Medical Officer
Center for Biologics Evaluation and Research
Office of Tissues and Advanced Therapies
U.S. Food and Drug Administration
Tel: 240-402-5564
Robert.Sokolic at fda.hhs.gov<mailto:Robert.Sokolic at fda.hhs.gov>
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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, December 29, 2016 4:35 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] LRBA and severe Thrombocytopenia with chronic CMV and EBV infection
Dear Mehdi,
Only 2 patients: I tried RTX in two patients with LRBA def. (one AIHA and one ITP) and worked fine (cytopenias and splenomegaly solved). In the first patient a late relapse ended in splenectomy as response to RTX was too slow.
It seems to work, but be ready for retreatment
Hope this helps
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
2016-12-29 10:10 GMT+01:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:
Dear Colleagues
I have a 7 years old male patient with LRBA deficiency , Chronic CMV and EBV infection.
Developed severe thrombocytopenia ( PLT : 1000 ) did not respond to high dose IVIg ( 2 gm/kg) in the last week , developed wet bleeding , received PLT transfusion .
Other cell lines are within Normal range except low ALC ( 1.5) for years . His CMV PCR is 309 copies and EBV : 4000 copies , Val gancyclovir was stopped last week with no improvements on PLT count.
He was on Plaquinil (Hydroxy Chloroquien : 7 mg /kg/ day , but stopped by the family 2 months back .
Abatacept<https://www.google.com/url?sa=t&rct=j&q=&esrc=s&source=web&cd=1&cad=rja&uact=8&ved=0ahUKEwi4pq-lg5nRAhUIIsAKHTkHAc8QFggZMAA&url=https%3A%2F%2Fen.wikipedia.org%2Fwiki%2FAbatacept&usg=AFQjCNGoIziW23fB8bVrpNH7JC-ULIR1_g&bvm=bv.142059868,d.d2s> is not available in Qatar.
We will do Bone Marrow Aspiration before starting Corticoids .
My questions:
Any previous experiences in giving Rituximab?
Which one is preferable Corticoids or Rituximab ?
Your kind advice is highly appreciated .
Mehdi Adeli,
MD, FAAAAI, FACAAI, FAAP, A.B.A.I, A.B.P
Sr. Consultant Allergy & Immunology
Hamad Medical Corporation
Sidra Medical And Research Center
Tel (O) :( 974) 4439-0328
Mob : (974) /33429669.
Fax: (974) 4439-7857
E-Mail: madeli at hamad.qa<mailto:madeli at hmc.org.qa>
Web : http://aiap.hamad.qa<http://aiap.hamad.qa>
Research Gate : http://www.researchgate.net/profile/Mehdi_Adeli
ORCID ID: http://orcid.org/0000-0002-3051-3080<http://orcid.org/0000-0002-0346-5570>
Doha- Qatar
________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Thursday, December 22, 2016 10:39 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Severe recurrent sinusitis in a CVID pregnant women
I have used ATB combination in the past with success, I will certainly suggest this combination to her OB. Thank you very much, best regards
Alejandro Malbran
________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Thursday, December 22, 2016 2:08 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Severe recurrent sinusitis in a CVID pregnant women
You could certainly increase her dose but, if she has had prolonged treatment with a beta-lactam, I would consider trimethoprim/sulfa (until 36 weeks) or clarithromycin. A nasal endoscopy may be helpful.
Richard Wasserman
On Thu, Dec 22, 2016 at 10:35 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Thank you very much for your answer.
Surgery once, five years ago. IgG levels right before IVIG infusion last month. Pregnant, no CAT scan possible right now. She does reasonably well on rifampin / amoxicillin / clavulanate + nasal steroids + nasal washings. But now she is pregnant and I can not find an appropriate treatment for her. Would anybody increase the IVIG dose, would it be useful to use any nasal
medication (beyond mupirocin already done). What about nasal IVIG, any thoughts? Experience?
Alejandro Malbran
________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Thursday, December 22, 2016 12:34 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Severe recurrent sinusitis in a CVID pregnant women
Has she ever had sinus surgery? When was her last sinus CT and what did it show? When was she last completely well? What was her IgG level at that time?
Richard Wasserman
Dallas
On Thu, Dec 22, 2016 at 9:15 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Dear Sir,
I would greatly appreciate your input to treat a young lady, 16 weeks pregnant, with severe sinusitis. She does nasal washing and takes amoxicillin. Her IgG is 975, no IgA, IgM nor IgE. She is replaced every 4 weeks with 30 gr. Any suggestion is welcome.
Best regards,
Alejandro Malbran
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