[PAGID] Autoimmune neutropenia

[Ashish Kumar]

Chris,

You described a typical case of autoimmune neutropenia.  AIN can last a 
long time and I don't think G-CSF is doing much other than making us 
feel better when we temporarily fix his neutropenia. You could give him 
all the G-CSF you want, but as soon as you stop it, his neutropenia will 
recur. The bone marrow findings are also typical of AIN. I have a hard 
time chalking the sepsis/osteomyelitis to AIN, and it may be the other 
way around. MRSA osteomyelitis can occur with a normal neutrophil count too.

I have a patient who had sever AD and FTT, who had eosiniphilia, high 
IgE, and initially also had lymphocytosis - ALC of 24k, mostly T-cells. 
He too had normal FOXP3 expression with relatively high number of 
CD25+/FOXP3+ cells and every other immune testing was negative as well. 
I found him to have a heterozygous RAG-2 mutation, one that hasn't been 
described before. No one knows if this was a red herring or not, but my 
patient is also African-American with a strong family history of atopic 
dermatitis. He also tested positive for all food allergies. I tried 
several topical steroid preparations for his skin, none worked and he 
had chronic watery diarrhea that kept him from gaining weight. At 10 
months, he weighed 4.5 Kg. Systemic steroids made him a new kid in 1 
week. When I tapered the steroids, his skin and diarrhea flared again, 
so I have initiated a very slow wean. He has finally gained some weight. 
I now ignore the food allergy tests in these patients and rely on 
challenge which typically allows resumption of a normal diet.

Ashish

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

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Chris Seroogy wrote:

> 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

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