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

[dmvascon at usp.br]

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 

----- Mensagem original -----


> De: "Javeed Akhter" <javeed.akhter at advocatehealth.com>

> Para: "CIS-PIDD" <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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