[CIS PIDD] [cis-pidd] LRBA and severe Thrombocytopenia with chronic CMV and EBV infection

Thu Dec 29 04:34:31 EST 2016

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. *0034**606732959 / * 0034913908569  /  Fax 0034913908772
<0034934893039>luisignacio.gonzalez at salud.madrid.org
<luisignacio.hdoc at salud.madrid.org>ORCID ID:  orcid.org/0000-0001-6917-8980
Researcher ID: B-9257-2009ResearchGate:
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>:

>
>  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 <madeli at hmc.org.qa>*
>
> *Web : http://**aiap.hamad.qa <http://aiap.hamad.qa>*
>
> *Research Gate : http://www.researchgate.net/profile/Mehdi_Adeli
> <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>
> *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>
> *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>
> 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>
>> *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>
>> 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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>>
>>
>>
>> --
>> 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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>
>
>
> --
> 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
>
> ---
>
> You are currently subscribed to cis-pidd as: amalbran31 at hotmail.com.
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