[PAGID] digeorge with lymphoid hyperplasia?
Kathleen E. Sullivan
sullivak at mail.med.upenn.edu
Wed Oct 24 15:14:56 EDT 2007
I would also think of ALPS. If so- that will guide your therapy.
Kathleen E. Sullivan MD PhD
Chief, Division of Allergy and Immunology
Professor of Pediatrics
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363
On Oct 24, 2007, at 3:11 PM, Ashish Kumar wrote:
> I just saw a 13 year old girl who had a VSD closure at birth, and was
> diagnosed with ITP 2 years ago. She now has mediastinal and axillary
> lymphadenopathy and massive splenomegaly (spleen almost into
> pelvis). I
> initially thought she had lymphoma because her LDH and uric acid were
> elevated. But a lymph node biopsy only showed follicular
> hyperplasia. The
> chest CT also showed a right sided aortic arch and I thought she
> looked
> dysmorphic, but her calcium level is normal. Her absolute
> lymphocyte count
> is 1000 (which our lab labeled as normal but I think is low for a
> 13 year
> old). The absolute CD4 is 450 and absolute CD8 count is 250. Also,
> IgA is 7,
> IgG is 620 (low for her age) and IgM is 250 (high for her age).
> Diphtheria
> and Tetanus titers are reactive, but low (both 0.07). She is
> clinically
> stable and has no history of recurrent infections.
>
> If she has DiGeorge (FISH pending); why the lymphoid hyperplasia?
> Or is her
> diagnosis CVID? I don't have her CD27 count.
> She doesn't have autoimmune hemolytic anemia or ITP (platelet
> counts are now
> normal). I found 2 reports of B cell lymphoma in DiGeorge patients,
> both EBV
> associated, but her lymph node stained negative for EBV.
>
> Any suggestions on what to do for the lymphoid hyperplasia - IVIG
> replacement won't help this, or would it?
>
> Ashish Kumar M.D., Ph. D.
> Assistant Professor
> Pediatric Hematology/Oncology/Blood and Marrow Transplantation
> University of Minnesota
>
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