[CIS-PAGID] Help with consult

[Kate Sullivan]

Multiple intestinal atresia SCID has been associated with GVHD.  It seems that there is a good bit of variability in the phenotype but I would still err on the side of caution.

You can use the baseline mitogens to guide you but titrating immune suppression in those who are already immune compromised is a tough subject.  If she is very immune compromised, you might think about a combined BMT- bowel transplant.

Kate
Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


On Nov 3, 2011, at 6:39 PM, Chong, Hey wrote:


>  

> Hello All:

>  

> I have a 13 yo female with “combined immunodeficiency ” (lymphopenia, with low CD4 count, low IgG and a protein losing enteropathy).  She had gastroschisis and atresias at birth now s/p numerous small and large bowel resections currently being evaluated for small bowel/colon/stomach transplant.   The transplant team would like to know what to expect with the immunosuppression used for transplantation in the setting of her immunodeficiency.  Could anyone comment specifically on the role of immunodeficiency in GVHD and infections post transplant?

>  

> Does anyone have any experience with managing transplants in CVID patients and what to expect?  Should they be monitored any differently?  Should the immunosuppression used be any different than any other patient?

> .

> More history:

> CID diagnosed in 2006

> Poor/absent response to vaccines

> Low IgG but normal IgA and IgM in the past currently on SQ IGx 2 years

> Decreased T cell proliferation with mitogens and antigens

> Lymphopenia with CD4 counts as low as 70 and history of PCP prophylaxis without hx of OI's

> Last 2 months with splenomegaly

> adrenal insufficiency

>  

>  

>  

> I would appreciate any help and advice and willing to answer any questions. 

> 

> Thanks very much

>  

> Hey Chong

>  

>  

> Hey Jin Chong MD PhD

> Assistant Professor of Pediatrics

> Division of Pulmonary Medicine, Allergy & Immunology

> Children's Hospital of Pittsburgh of UPMC

> One Children's Hospital Drive

> 4401 Penn Avenue

> Pittsburgh, PA 15224

> tel 412-692-7885

> fax 412-692-8499

> 

>  

>  

>  


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