[PAGID] lymphopenia

Tue Jun 20 14:27:09 EDT 2006

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org]On Behalf Of Christine Seroogy
Sent: Tuesday, June 20, 2006 11:20 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] lymphopenia

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 <mailto:baker084 at tc.umn.edu> 

  _____  

From: pagid-bounces at list.clinimmsoc.org
<mailto:pagid-bounces at list.clinimmsoc.org>  [
mailto: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 <mailto: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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