[PAGID] patient

[K. Scott Baker]

Unusual case, what about JMML?  Marrow findings can be very difficult to
interpret.  

 

K. Scott Baker, MD, MS

Pediatric Blood and Marrow Transplant Program

University of Minnesota

  _____  

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Christine Seroogy
Sent: Thursday, October 12, 2006 1:09 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] patient

 

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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