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

[Akhter, Javeed]

Thanks
Will read the article.  She did meet the ESID criteria for "probable CVID."
javeed

From: Dr. Esther de Vries [mailto:esid at estherdevries.nl]
Sent: Thursday, May 30, 2013 1:35 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] 17 yr old WF with CVID and neutropenia

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<mailto: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


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