[CIS PIDD] [cis-pidd] TC history
Sullivan, Kathleen
sullivak at mail.med.upenn.edu
Thu Jan 29 16:18:43 EST 2015
I might observe a smidgen more but the RO excess certainly does not bode well.
Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363
> On Jan 29, 2015, at 4:02 PM, Haines, Kathleen M.D. <KHaines at HackensackUMC.org> wrote:
>
> Dear All: Al Gillio asked me to post this patient on the list-serve. Basically it’s an infant who failed newborn screening but appears to have good cell function. Transplant? Or not? His summary follows.
>
> Kathleen A. Haines, MD
> Section Chief, Pediatric Immunology
> Section of Pediatric Rheumatology and Immunology
> Joseph M. Sanzari Children's Hospital
> HackensackUMC
> 30 Prospect Ave.
> Hackensack, NJ 07601
>
> Tel: 551-996-5306
> Fax: 201-996-9815
> email: khaines at hackensackUMC.org
>
>
> 11/12/14: Notified NJ NB SCID screen Trec CT 41.1
>
> 11/13/14: First visit HackensackUMC, normal exam, normal CBC, no eosinophilia, no rash
> ALC 1320, CD3 119, CD4 53, CD8 52, CD56 594, CD19 26
> CD4, CD45 RA 46%
> CD4, CD45 RA 54%
>
> HIV-1 RNA PCR Negative
> Possible T-B-NK + SCID put on strict isolation at home
>
> 11/17/14: Mayo mitogens: Viability 43.3 (>75)
> PHA % CD45 30.7 (>49.5)
> PHA % CD3 49 (>58.5)
>
> 11/19/14: FISH PB XY only – no maternal engraftment
>
> 12/02/14: Mutation analysis for RAG 1, RAG 2, DCLRE1C negative
>
> 1/07/15: ALC 627, CD3 131, CD4 88, CD8 31, CD56 245, CD19 19
> CD4, CD45 RA 18%
> CD4, CD45 RO 79%
> IgG 434, IgM 17, IgE <2, IgA <5
> Trec 839 x 106 CD3 cells
>
> Mayo mitogens: Viability 62.4 (>75)
>
> PHA % CD45 31.4 (>49.9)
> PHA % CD3 41.8 (>58.5)
>
> Viability 58.5 (>75)
>
> aCD3 aCD28% CD45 49.1 (>37.5)
>
> aCD3 aCD28% CD3 67.0 (>47.6)
>
>
> 1/21/15: CXR: Normal, no effusion, normal heart size, no obvious thymus
> AFP: 48.8 (normal for age), IgG 371
> Microarray sent to r/o DiGeorge and other syndromes
> Complete abdomen ultrasound to r/o third space fluid and abnormal kidney (scheduled)
>
> Echocardiogram (scheduled)
> Gaining weight, no infections, normal exam. Still on isolation at home.
>
>
>
>
> In summary:
> 1. Abnormal Newborn Screen
> 2. Decreased ALC, decreased CD3, decreased CD4, decreased CD8, decreased CD19, normal CD56
> 3. <80% CD4, CD45 RO
> 4. Almost NL PHA and anti CD3/anti CD28 mitogen assays
>
> Questions:
> 1. Does not fit PIDTC definition of classic SCID or leaky SCID but consistent with idiopathic <> T-cell lymphopenia (JAMA, 2014)
> 2. Definitive treatment?
> · Observation and viral and PCP prophylaxis and IVIG vs. BMT
> 3. Further studies?
> · Complete SCID mutation analysis
> · BM Aspiration? R/O leukemia
>
>
> __________________________
> Alfred P. Gillio, MD
> Co-Director
> Institute for Pediatric Cancer & Blood Disorders
> Joseph M. Sanzari Children’s Hospital
> HackensackUMC
> 30 Prospect Ave, WFAN Bldg., 1st floor
> Hackensack, NJ 07601
> 551-996-5600
> agillio at HackensackUMC.org <mailto:agillio at HackensackUMC.org>
>
>
> Kathleen A. Haines, MD
> Section Chief, Pediatric Immunology
> Section of Pediatric Rheumatology and Immunology
> Joseph M. Sanzari Children's Hospital
> HackensackUMC
> 30 Prospect Ave.
> Hackensack, NJ 07601
>
> Tel: 551-996-5306
> Fax: 201-996-9815
> email: khaines at hackensackUMC.org
>
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