[PAGID] lymphopenia

[Kathleen E. Sullivan]

I have seen two cases of Castleman's with what we are pretty sure was a 
secondary immunodeficiency (ALC normal prior to symptoms).  I am very 
sad to report, both patients died after traditional chemotherapy.

On Jun 20, 2006, at 2:20 PM, Christine Seroogy wrote:

> 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
>>
>>  
>>
>>
Kathleen E. Sullivan MD PhD
Chief, Division of Allergy and Immunology
Associate Professor of Pediatrics
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 215-590-3044
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