[CIS PIDD] [cis-pidd] 17 yr old WF with CVID and neutropenia

[Dr. Esther de Vries]

Dear Javeed Akhter, is her diagnosis really CVID? 
We recently published a very similar case (no neutropenia so far, otherwise very much the same clinical course): Case Reports in Immunology Volume 2012 (2012), Article ID 196417, 6 pages, doi:10.1155/2012/196417. 
Best regards, dr. Esther de Vries, Consultant in Pediatric Infectious Diseases & Immunology, Jeroen Bosch Hospital, 's-Hertogenbosch, the Netherlands

Op 29 mei 2013, om 19:31 heeft "Akhter, Javeed" <javeed.akhter at advocatehealth.com> het volgende geschreven:


> Hi friends

> I need your input with a 17 yrwf with CVID diagnosed approximately 10 yrs ago. Here is a brief summary.

> ·        Her presentation was of Evan’s syndrome early in life

> ·        At 7 or 8 yrs of age she had a LLL pneumonia and on imaging multiple small nodules in the lung parenchyma as well as mediastinal lymphadenopathy.  Both the mediastinal nodes and the pulmonary nodules revealed non-caseating granulomas.  This is when I saw her and diagnosed CVID (probable according to the ESID criteria)

> ·        She was also noted to have splenomegaly that has increased in size and is currently 17.5 cm linear measurement on ultrasound

> ·        She was started initially on IVIG and now SCIG weekly with an additional dose of IVIG every 2-3 months to keep the IgG lvels high (empiric therapy following personal communications with dr Charlotte Cunningham-Rundles).  She was also started on po steroids initially daily and currently qod.  She is down to 10 mg qod

> ·        At around 12 years of age her thrombocytopenia became severe and nonresponsive to increased steroids therefore she was given four doses of Riuximab.  She responded well to it.

> ·        With IG replacement and steroids and possibly Rituximab she has done well including with her lung disease that is almost completely resolved

> ·        Last week she came in with a viral syndrome, low grade fever, aches and pains and severe neutropenia with an absolute count of 100-200.  She was given three doses of Nuprogen with no response.  This is the first time she has had severe neutropenia.  NP swab for viral PCR is negative.

> ·        There is no thrombocytopenia or anemia.  The lung function and xray are fine. No other new physical findings.  She looks healthy

> What should be my next step

> ? bone marrow biopsy

> ? more Nuprogen

> ? blast of steroids, oral or pulse

> ? and finally what is your opinion on splenectomy on this young woman

>  

> Thank you much

>  

> Javeed Akhter, M.D.

> Section Head

> Pediatric Pulmonology

> Allergy and Immunology

> Director

> Cystic Fibrosis Care Center

> JMF Immunology Referal Center

> p 708.684.5810

> F 708.684.2045

>  

> 

> This e-mail, and any attachments thereto, is intended only for use by the addressee(s) named herein and may contain legally privileged and/or confidential information. If you are not the intended recipient of this e-mail (or the person responsible for delivering this document to the intended recipient), you are hereby notified that any dissemination, distribution, printing or copying of this e-mail, and any attachments thereto, is strictly prohibited. If you have received this e-mail in error, please respond to the individual sending the message and permanently delete the original and any copy of any e-mail and any printout thereof.

> ---

> 

> The CIS-PIDD listserv is supported by:

> 

> 

> The science & practice of human immunology

> 

> P: +1.414.224.8095

> E: info at clinimmsoc.org

> 

> Not a member of CIS? Please visit www.clinimmsoc.org to join!

> 

> You are currently subscribed to cis-pidd as: esid at estherdevries.nl.

> To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824446.14623d29c55a14564e580980e6a78208&n=T&l=cis-pidd&o=43414022

> 



---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=43417704
or send a blank email to leave-43417704-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20130530/d6bf4f3d/attachment.htm>