[CIS PIDD] [cis-pidd] Test for OKT4 epitope deficiency

[CIS-PIDD]

Dear Araceli,

Does the patient have a large number of CD8-CD3+ cells (total CD3 count – CD8 count).  If so it might indicate that the antibody isn’t binding to CD4, you could ask around to some of the clinical labs to see who uses antibodies other than OKT4 to bind CD4 (it would also be very easy for any lab-based folks around you to look at).  The history of severe aplastic anemia and poor proliferation is certainly concerning for a PID - does the patient express class II?

I have a patient with PI3KCD GOF who presented with severe aplastic anemia and skewed CD4:8 ratio.

Best,

Megan


Megan A. Cooper, MD, PhD
Assistant Professor, Department of Pediatrics
Division of Rheumatology
Washington University School of Medicine
Cooper_m at kids.wustl.edu<mailto:Cooper_m at kids.wustl.edu>
Lab website: http://research.peds.wustl.edu/Default.aspx?alias=research.peds.wustl.edu/Labs/Cooper_M
(lab office) 314-286-0262
(lab fax) 314-286-2895



From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Monday, October 24, 2016 3:33 PM
To: CIS-PIDD
Subject: [cis-pidd] Test for OKT4 epitope deficiency

Dear Professors,

I follow a patient whom I would like to test for OKT4 epitope deficiency. Does any lab perform this test in the US?
The patient is a 2yo male who presented with severe autoimmune aplastic anemia and was found to have severe CD4 lymphopenia (other lymphocyte subsets are normal) and asymptomatic CMV viremia. Infection history is otherwise unremarkable and patient otherwise appears healthy. HIV and TB tests are negative. He had low response to PHA and ConA and normal response to Pokeweed. IgG is nl, IgM is slightly low. He got IVIG back in July before I was consulted, I haven't gotten vaccine titers.
Aplastic anemia did not improve once CMV viral load became negative s/p treatment with valacyclovir.
I'm considering sending SCID genetic panel on this patient as well.

Thank you,

Araceli

Araceli Elizalde, MD
Immunology Clinic Director
Assistant Professor of Clinical Pediatrics
Allergy, Immunology & Rheumatology
Children's Hospital of San Antonio
Baylor College of Medicine


-----Original Message-----
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Sent: Fri, Oct 21, 2016 5:37 pm
Subject: [cis-pidd] Steroid-responsive Dysuria in CGD
Colleagues:

I follow an 18 month old boy with X-CGD.  He seems to have dysuria that is responsive within a day or two to systemic steroids.  He has had a normal procto-colonoscopy and most recently normal cystoscopy with biopsies negative for inflammation and with no visual evidence of obstruction in the lower urinary tract.

What am I missing?

Joe Church
Children’s Hospital Los Angeles

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