[PAGID] EBV negavtive lymphomatoid granulomatosis

[Ashish Kumar]

I have a 15 year old boy who presented with recurrent headaches, 
vomiting and right sided weakness about a year ago. MRI of brain showed 
multiple discrete lesions. Chest CT also showed nodules which on biopsy 
showed angiocentric polyclonal lymphoid infiltrate i.e. lymphomatoid 
granulomatosis (LYG). EBER stains were negative as was his peripheral 
blood PCR was EBV. Published reports also show that CNS LYG is often 
EBV-negative. His symptoms improved on their own so nothing was done. 
There was no family history suggestive of XLP, his lymphocyte subsets 
and Ig levels were all normal and he was a previously healthy, well 
developed young man. Family declined XLP testing. About a month ago, he 
started having symptoms again and repeat MRI of brain showed numerous 
new lesions. So, I elected to treat him with steroids - 2 mg/Kg. He 
didn't have symptoms for 2 weeks, but then started having them again - 
headache and vomiting. I am getting another MRI today, but regardless of 
what it shows, I think he needs more therapy and I was going to add 
Rituximab. Any other recommendations? There are reports of using full 
lymphoma based chemotherapy but I'd like to avoid that if possible. I am 
going to persuade the family to allow XLP testing as well. Any ideas or 
suggestions would be helpful.

Thanks!
Ashish

-- 
Ashish Kumar, MD, PhD
Assistant Professor of Pediatrics
Division of Hematology/Oncology/Blood and Marrow Transplantation
University of Minnesota

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