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

Thu Dec 29 04:10:49 EST 2016

 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
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>
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<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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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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