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

[Daniel Suez]

I will also consider to look for GATA2 deficiency.  I am not sure if that
was brought up.
Dan Suez


Allergy, Asthma & Immunology Clinic PA

Daniel Suez, MD
Board Certified in Allergy & Immunology and
Diagnostic Laboratory Immunology
Clinical Associate Professor
UT Southwestern Medical School at Dallas
Allergy & Immunology Division
1115 Kinwest Pkwy, Suite 100
Irving TX 75063
T: 972-401-0545  C: 214-708-0727




On 5/30/13 9:14 AM, "Akhter, Javeed" <javeed.akhter at advocatehealth.com>
wrote:


>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/k

>umar/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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