[PAGID] lymphopenia

[Jennifer Puck]

I have seen instances of lymphomas called Castleman's that were 
subject to disagreement upon review. I would suggest having the case 
reviewed at NIH by Dr. Elain Jaffe. Castleman's is supposed to occur 
in individuals at advanced age.
Dr. Jaffe will accept referrals pathologist-to-pathologist and her 
expertise is outstanding. You can email her at Elaine S Jaffe 
<ej7n at nih.gov>.
Jennifer Puck

>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
>>
>><mailto:baker084 at tc.umn.edu>baker084 at tc.umn.edu
>>
>>
>>From: 
>><mailto:pagid-bounces at list.clinimmsoc.org>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: <mailto:pagid at list.clinimmsoc.org>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


-- 


Jennifer M. Puck, M.D.                      	  

New position and contact information:

Professor of Pediatrics
University of California, San Francisco
Department of Pediatrics, Box 0519
HSE 301A
513 Parnassus Ave.
San Francisco, CA  94143

Phone 415 476-3181
FAX    415 502-5127
puckj at peds.ucsf.edu
-------------- next part --------------
An HTML attachment was scrubbed...
URL: http://seven.pairlist.net/mailman/private/pagid/attachments/20060621/d48c27cc/attachment.html