[PAGID] patient

[Christine Seroogy]

Dear Colleagues:

I have a perplexing case that was a second opinion and wonder if any  
of you have seen a similar presentation.  This is a 2y/o boy with  
recurrent rashes (urticarial--angioedema and eczematoid),  
hepatosplenomegaly and lymphadenopathy since age 6 months.  The  
rashes are fleeting and this has been a non-issue for over 6 months  
but the other clinical findings are persistent. No fevers or weight  
loss.  He was evaluated at another institution and found to have  
normal B and T cell numbers and function.  Liver biopsy demonstrated  
extramedullary hematopoiesis.  CBC with anemia and marked  
microcytosis.  Repeat abdominal CT redemonstrated HSM and abnormal  
bone signal suggestive of an infiltrative process and enlarged  
diffuse lymph nodes.  Bone marrow full of hematogones and trilineage  
hematopoiesis and markedly diminished Fe stores and no evidence of  
malignancy.  His immune evaluation here consisted of ALPS panel and  
SAP flow  (both done in Cincinnati.) Flow at our institution  
demonstrated increased numbers of peripheral B cells (abs. CD19  
3700)  Studies done in Cincinnati showed normal SAP and the ALPS  
panel did not fulfill the criteria, but interestingly he has low % of  
memory B cells (CD27+:  5.6%) and increased % CD5 B cells (52%).  So  
it seems his B cell compartment is abnormal but the HSM and  
extramedullary hematopoiesis is unusual.  Infectious etiologies have  
been looked for and unrevealing.  His presentation is difficult to  
unify.  I welcome any suggestions or comments.  Regards, 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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