[PAGID] Autoimmune neutropenia

[Chris Seroogy]

Dear Colleagues,

I would appreciate any thoughts on an enigmatic 10m/o African American boy
followed at our institution for multiple medical problems.  He was born term
and well except for mild atopic dermatitis until Jan. of this year. At that
time he presented with MRSA sepsis/osteomyelitis (hip) and neutropenia.  His
bone marrow showed increased myeloid precursors and trilineage
hematopoiesis.  Extensive immune evaluation negative.  The neutropenia was
felt to be secondary to his infection felt to be caused by breakdown of his
integument.  However, the neutropenia has persisted to the point that our ID
folks started GCSF.  I was consulted some months later to revisit his immune
system since he had and influenza A infection and history of recurrent URTI.
There is a strong family history of atopic and this child has severe AD and
probable clinical food allergy with FTT thought to be secondary to poor
intake.  We have optimized his diet and he is making slow, but steady gains
in his weight.  Development is not a concern.
Notable data:

Repeat flow, antibody levels and titers, CH50, HIV all negative/normal.
IgE 2000 with multiple food sensitivities
Foxp3 detactable and high percentage in CD25+
ALPS panel not indicative of ALPS, but high % B22-+DNTC (24.7%)
Antineutrophil antibodies strongly positive (>3SD and run at ARUP)

No enlarged liver or spleen.  We held his GCSF and repeated marrow--it was
difficult to interpret even 4 days off GCSF with a tremendous number of
hematogones, trilinear hematopoiesis and mostly mature granulocytes in the
granulocytic lineage.

We tried high-dose IVIG thinking that might give a respite from GCSF and
this was without success.  His neutrophils have been consistently below 500
while holding GCSF now.  He is being admitted for fever with an ANC 180.

My question is if this is a phenotype that others have seen?  Any
recommended additional testing or therapeutic interventions?  Thank you for
your opinions, Chris


Chris Seroogy, M.D.

University of Wisconsin

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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