[CIS PIDD] [cis-pidd] WAS question

[CIS-PIDD]

Dear colleagues,

We have a strange WAS case that we wanted to run by you all.

We have a 5 month old with WAS (brother effected, genetically confirmed: WASP: p.Q20X) who was noted to have profound neutropenia in March (ANC consistently <100).  He was initially treated with steroids at UVA but had no response.   We admitted him in early April, and he also had a moderate DAT+ hemolytic anemia which leveled off after rituxan therapy.  However, his ANC did not improve.  He is now admitted with febrile neutropenia (and found to have a pseudomonas central line infection), and bone marrow evaluation today showed markedly left shift with only immature neutrophils, consistent with maturation arrest.  Flow is pending.

Has anyone had similar experiences?  We are working toward HSCT on the sooner side, but are very puzzled by this finding.

Thanks in advance,
Mike



Michael D. Keller, MD

Assistant Professor, Division of Allergy / Immunology

Center for Cancer and Immunology Research

Jeffrey Modell Diagnostic and Research Center for

Primary Immunodeficiency Disorders

Children's National Health System

111 Michigan Ave NW, M7745A

Washington, DC 20010

Clinic: 202.476.3016

Office: 202.476.5843

Fax: 202.476.2280

www.ChildrensNational.org

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