[PAGID] patient

Wed Oct 25 12:49:16 EDT 2006

Hi,

Our allergy/immunology fellows discussed this case and had the following
thoughts that may help:

"The constellation of symptoms are perplexing, and there may have
already been an extensive workup done which was not listed that may have
come back as being negative or noncontributory to the patients
condition. 

The following is a list of thoughts that come to mind when examining the
case: Approaching the case from a findings/symptomatology approach and
stratifying the most significant to least may help. This approach may
better allow us to assess the problem. For example, the
hepatosplenomegaly and extramedullary hematopoeisis should be the first
issues to be  addressed. A primary immunodeficiency is unlikely since
there is no evidence of infection, autoimmunity or malignancy. If these
conditions have been ruled out, then the anemia is probably  the central
issue. What is the origin of the anemia? Are the microcytes spherocytes
or sickle cells? Is it related to iron stores or metabolism? Is the
trilineage hematopoiesis an early stage of myelofibrosis? 

The skin rashes may be related to the underlying condition or to a
concomitant atopic condition.

Best regards,

Ricardo Sorensen

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of K. Scott Baker
Sent: Saturday, October 21, 2006 11:11 AM
To: pagid at list.clinimmsoc.org
Subject: RE: [PAGID] patient

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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