[CIS PIDD] [cis-pidd] Neutropenia in XLA

[CIS-PIDD]

Although neutropenia in XLA is well known, the mechanism is not that well understood. Published literature supports the association with infections, but we have seen it independent of severe infections, and the ANC can go down to 0. Viral infections are definitely possible, as is a T-lymphoproliferative process. Response to Filgrastim is definitely reassuring. If he is not having recurrent fevers, stomatitis or infections, I would just observe for now.

Ashish
Ashish Kumar, MD, PhD
Associate Professor of Pediatrics
Director, Langerhans Cell Histiocytosis Center
Director, Pediatric Hematology/Oncology Fellowship Program
Cincinnati Children's
3333 Burnet Avenue, , Cincinnati, OH 45229

[https://www.cincinnatichildrens.org/-/media/295b830c6eb94ee2b662c61a2945e5e6.ashx?h=67&w=200]<https://www.cincinnatichildrens.org/>

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Friday, October 13, 2017 2:26 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re: [cis-pidd] Neutropenia in XLA

Is there any involvement of other hematologic lineages? Has a bone marrow aspiration or biopsy been done?
I am not aware of isolated neutropenia in XLA, but have seen this in the absence of anemia in an X-HIM patient that was eventually attributed to chronic parvovirus B19 infection. PCR could detect viremia, parvovirus inclusions in marrow biopsies will corroborate the diagnosis and in situ hybridization can confirm the infection.

All the best,
Soheil Chegini

________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Sent: Friday, October 13, 2017 1:51 PM
Subject: [cis-pidd] Neutropenia in XLA

Dear all, we have a 13 moths old boy with genetically confirmed XLA. At diagnosis he presented recurrent URTI, agammaglobulinemia and neutropenia. Despite immunoglobulin replacement therapy for 5 months with correct IgG levels (Ig G = 1300 mg/dL), mild to moderate neutropenia persists requiring intermittent G-CSF administration. Do you have any experience with long-term neutropenia in XLA? As far as I know neutropenia occurs at the time of diagnosis and it resolves after IgRT is started and normal IgG trough levels achieved.

Best regards from BCN,

P.



Pere Soler Palacín, MD, PhD, MSc.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Vall d'Hebron Research Institute (VHIR)
Assistant Professor. Universitat Autònoma de Barcelona (UAB)
Director of the Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies (Barcelona)
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Catalonia.
Tel. 0034934893140  /  Fax 0034934893039
psoler at vhebron.net<mailto:psoler at vhebron.net>  /  34660psp at comb.cat<mailto:34660psp at comb.cat>
Web: www.upiip.com<http://www.upiip.com>
My ORCID
ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin
LinkedIn: www.linkedin.com/in/pere-soler-palacin<http://www.linkedin.com/in/pere-soler-palacin>


No imprimir aquest correu ajudarà a preservar el medi ambient.

Si vostè no és el destinatari del missatge, o l'ha rebut per error, si us plau notifiqui-ho al remitent i destrueixi el missatge amb tot el seu contingut. Està prohibida la distribució no autoritzada del contingut d'aquest missatge.

No imprimir este correo ayudará a preservar el medio ambiente.
Si usted no es el destinatario del mensaje, o lo ha recibido por error, notifíquelo por favor al remitente y destruya el mensaje con todo su contenido. Está prohibida la distribución no autorizada del contenido de este mensaje.
---
You are currently subscribed to cis-pidd as: schegini at yahoo.com<mailto:schegini at yahoo.com>.
To unsubscribe click here: http://cts.dundee.net/u?id=96396879.1c4ba390e5cba8b441ad14c59c207858&n=T&l=cis-pidd&o=4615971
(It may be necessary to cut and paste the above URL if the line is broken)
or send a blank email to leave-4615971-96396879.1c4ba390e5cba8b441ad14c59c207858 at lyris.dundee.net<mailto:leave-4615971-96396879.1c4ba390e5cba8b441ad14c59c207858 at lyris.dundee.net>


---

You are currently subscribed to cis-pidd as: ashish.kumar at cchmc.org<mailto:ashish.kumar at cchmc.org>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396410.80d0426a8f030c7d59f25e5e3c6ac047&n=T&l=cis-pidd&o=4616014

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4616014-96396410.80d0426a8f030c7d59f25e5e3c6ac047 at lyris.dundee.net<mailto:leave-4616014-96396410.80d0426a8f030c7d59f25e5e3c6ac047 at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4616058
or send a blank email to leave-4616058-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20171013/51fb946e/attachment-0001.html>
-------------- next part --------------
A non-text attachment was scrubbed...
Name: image001.png
Type: image/png
Size: 2170 bytes
Desc: image001.png
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20171013/51fb946e/attachment-0001.png>