[CIS PIDD] [cis-pidd] LGL and CVID

[Sullivan, Kathleen]

I am no expert with an n=2 but here are my 2 cents.  The fist child presented very young and was well treated for a period with MTX.  His spleen became huge over a few years and was taken out.  Once that happened, his disease became more aggressive.  IT was as if the spleen was sort of the sink for the cells and with out that , they began to infiltrate everything else.  In any case, he had a BMT and did very well.

Second case is ongoing.  XLA patient with LGL and primarily musculoskeletal disease.  He has been over the years treated with everything but alpha IFN worked the best.  He was taken off for side effects but in terms of controlling his disease, it was the best by far.  HIs LGL disease is now over 5 years old.  He is homebound and can't do anything.  He has had to defer his college admission and has a terrible quality of life.  He would be a high risk BMT, but I truly think it is the only thing that holds much hope for him.




On Apr 1, 2013, at 7:59 PM, Maite de la Morena wrote:


> Dear all

> Would appreciate insights/experience with management of a complex 27 year old female who has carried a diagnosis of CVID since age 14 year now with diagnosis of large granular lymphocyte leukemia (LGL) presenting with pure red cell aplasia no neutropenia. Oncologists started steroids and discussing Cytoxan/Mtx/CyA

> Thank you

> Maite

>  

> Maite de la Morena, MD

> Associate Professor of Pediatrics

> Division of Allergy and Immunology

> University of Texas Southwestern Medical Center in Dallas

> 5323 Harry HInes Blvd

> Dallas, Texas 75390-9063

> Phone 214 456-5161

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> Email: maite.delamorena at utsouthwestern.edu

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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



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