[CIS-PAGID] Help with consult

[Ochs, Hans]

You have 2 problems to consider.

1.       As a SCID (associated with intestinal atresias, gene defect unknown), she may develop GvHD initiated by the lymphocyte rich intestinal graft, especially if the patient receives post transplant immunosuppression - as Kate pointed out. Tread slowly on additional immunosuppression.

2.       If she has significant cellular immune function (which I assume is the case having lived with SCID diagnosis for 13 years) she may reject the intestinal graft, unless you carry out drug induced immunosuppression that may interfere with point 1
This requires meticulous attention to both issues. You need to know her immune status prior to transplant and based on that, juggle the therapy to avoid these 2 transplant associated curses.
In vitro lymphocyte transformation to a mitogen, anti-CD3, specific antigens, T and B cell phenotyping, NK cell number and function (rejectionists!), neo antigen immunization (I assume she is on IVIG)
Challenging.
hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

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From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Chong, Hey
Sent: Thursday, November 03, 2011 3:39 PM
To: 'pagid at list.clinimmsoc.org'
Subject: [CIS-PAGID] Help with consult


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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