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

[Akhter, Javeed]

The spleen is firm and is palpable  about 5 inches below the costal margin
Thanks for the suggestion on looking for Histo on bone marrow.  I see a fair amount of it in the Chicago area
javeed

-----Original Message-----
From: Mel.Berger at cslbehring.com [mailto:Mel.Berger at cslbehring.com] 
Sent: Wednesday, May 29, 2013 11:03 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] 17 yr old WF with CVID and neutropenia

What is the size of her spleen on exam or scan ?   Bone marrow should have a touch prep and culture for Histo, which could certainly co-exist with CVID.



Mel Berger

________________________________
From: Akhter, Javeed [javeed.akhter at advocatehealth.com]
Sent: Wednesday, May 29, 2013 3:32 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] 17 yr old WF with CVID and neutropenia

Thanks

Javeed Akhter

On May 29, 2013, at 1:34 PM, "Kumar, Ashish" <Ashish.Kumar at cchmc.org<mailto:Ashish.Kumar at cchmc.org>> wrote:

I agree, you need a bone marrow biopsy to look for etiology, including partially treated lymphoproliferative disorders like HLH.

Ashish Kumar, MD, PhD
Cancer and Blood Diseases Institute
Division of Bone Marrow Transplantation and Immune Deficiency Cincinnati Children's Hospital Medical Center http://www.cincinnatichildrens.org/bio/k/ashish-kumar/
http://www.cincinnatichildrens.org/research/divisions/b/bone-marrow/labs/kumar/default/

From: dmvascon at usp.br<mailto:dmvascon at usp.br> [mailto:dmvascon at usp.br]
Sent: Wednesday, May 29, 2013 2:01 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] 17 yr old WF with CVID and neutropenia

Dear Dr. Javeed Akhter

I would try to detect the possible virus in this context.
Initially I would look for autoantibodies that could be relevant for neutropenia.
Afterwards I would get a bone marrow biopsy (maybe initially a myelogram) with PCR for myelotrophic viruses. Sometimes in the event of severe suppression of myeloid lineage the response to filgrastim is delayed.
If all negative I would think about steroids (pulse) or maybe mycophenolate mofetil.
If autoantibodies positive I would think about Rituximab again (with plasmaferesis??) I would not try to do splenectomy as CVID patients usually begin to present more severe infections.

Good luck with your patient

Dewton

Dewton de Moraes Vasconcelosm M.D.; Ph.D.
University of São Paulo School of Medicine

________________________________
De: "Javeed Akhter" <javeed.akhter at advocatehealth.com<mailto:javeed.akhter at advocatehealth.com>>
Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Enviadas: Quarta-feira, 29 de Maio de 2013 14:31:01
Assunto: [cis-pidd] 17 yr old WF with CVID and neutropenia 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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