[PAGID] lymphopenia

[Christine Seroogy]

Colleagues,

I have an update on the patient I presented last week and, again,  
welcome opinions in terms of management.  The pathology on the GI  
mass is now finalized as Castleman's disease.  Clinically, he does  
not fit into unicentric or multicentric, but falls somewhere in  
between.  It is CD20 negative and he is HHV8 positive.  So my heme/ 
onc colleagues are planning on treatment with chemotherapy vs.  
rituximab (even with known CD20 negativity); and are inclined toward  
rituximab therapy.  Given his underlying uncharacterized cellular  
immunodeficiency the move to BMT is being entertained after treatment  
of the Castleman's.  I don't think he has an allogeneic match and  
would require MUD.  Flow for SAP was normal. HIV testing has been  
negative.  I am wondering if any of you have seen Castleman's in your  
immunodeficient patients and if so what the treatment was and  
clinical course?  Thank you, Chris





On Jun 13, 2006, at 9:32 PM, K. Scott Baker wrote:

> We have had 2 cases in the last couple of years, the first ended up  
> having XLP, the second we have been unable to molecularly  
> characterize a defect, he has a “CVID” phenotype.  Interestingly  
> his lymphoma was metastatic Hodgkin’s at age 4, which recurred.  He  
> is being treated for the recurrence and then going to allo-HCT.   
> The XLP had several recurrences of his NHL (mainly abdominal  
> primaries).  He received non-myeloablative MSD BMT 2 yrs ago and  
> has had no evidence of his disease (or xlp) since.  Are you  
> planning on HCT for your patient?
>
>
>
> K. Scott Baker, MD, MS
>
> Pediatric Blood and Marrow Transplant Program
>
> University of Minnesota
>
> 420 Delaware St. SE, Mayo Bldg. Room D557
>
> Mayo Mail Code 484
>
> Minneapolis, MN 55455
>
> 612.625.4952  FAX 612.626.1434
>
> baker084 at tc.umn.edu
>
> From: pagid-bounces at list.clinimmsoc.org [mailto:pagid- 
> bounces at list.clinimmsoc.org] On Behalf Of Christine Seroogy
> Sent: Tuesday, June 13, 2006 1:51 PM
> To: pagid at list.clinimmsoc.org
> Cc: Kakumanu Sujani
> Subject: [PAGID] lymphopenia
>
>
>
> Dear Colleagues,
>
>
>
> I would like to get your opinions about a 15y/o boy was admitted to  
> our hospital with a gastrointestinal lymphoma (further  
> characterization pending.) He was initially seen in the immunology  
> clinic here at 4 years of age because of severe primary VZV  
> infection. Evaluation at that time demonstrated lymphopenia-- 
> predominately CD4--and poor mitogen response. His B cell numbers  
> and function is normal (measured by titers to various vaccine  
> antigens); he has been persistently hypergammaglobulinemic (IgG  
> 1000s and IgA 400s). NK cell numbers by CD16/56 slightly  
> diminished. HIV nonreactive. He developed a Burkitt's lymphoma  
> around age 10 and was successfully treated, he has had refractory  
> sinus disease and recurrent OM. No other infectious history or  
> autoimmune phenomenon. No relevant family history. I welcome any  
> thoughts on how to pursue a molecular diagnosis on this child or  
> hearing if you have seen similar cases. Thank you, Chris
>
>
>
>
>
>
>
>
>
> Chris Seroogy, M.D.
> Assistant Professor
> Dept. of Pediatrics
> Mail: H4/474 CSC, Mailstop 4108
> Shipping: H4/431 CSC, Mailstop 4108
> 600 Highland Ave.
> Madison, WI 53792
> phone: 608- 263-2652
> fax: 608-265-0164
>
>
>

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