[PAGID] lymphopenia

[dmvascon at usp.br]

We have seen 2 patients with primary CD4 lymphopenia that evolved with EBV
positive B cell lymphoma. 
The first presented several infections since childhood, improving after
pneumococcal vaccine for about 5 years. After that he developed "autoimmune"
hemolytic anemia (Mycoplasma +) and after 2 years a lung primary EBV + high
grade B cell non-Hodgkin lymphoma non-responsive to chemotherapy whose first
metastasis was in the brain.
The second presented disseminated warts (even in mucosal tissues) since
childhood that improved after alpha-interferon developing sicca syndrome
secondary to IFN. 
After 2 years he presented a cervical EBV + high grade non-Hodgkin lymphoma
treated with chemotherapy with complete remission. 
He received a HLA-identical BMT evolving with severe skin, hepatic and
intestinal GVHD dying after 80 days due to infection.

All the best,

Dewton

Dewton de Moraes Vasconcelos, M.D.; Ph.D.
Primary Immunodeficiencies Outpatient Unit - ADEE3003
Department of Dermatology
University of São Paulo Medical School


Citando Christine Seroogy <cmseroogy at wisc.edu>:

> 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
> 
>