[PAGID] Interesting case

[Paul Ogershok]

Patient had a Body PET/CT scan that just showed hepatosplenomegaly.

>>> sullivak at mail.med.upenn.edu 10/26/2005 4:46:55 PM >>>
I would definitely worry about parvo but if negative, I might think 
about thymoma.

On Oct 26, 2005, at 4:41 PM, Paul Ogershok wrote:

> I just saw a 18 yo with diagnosis of red cell aplasia and
> hypogammaglobulinemia diagnosed at age 14.  He was never sick prior
to
> age 14 and no family history of immunodeficiency.  Red cell aplasia
> responded to cyclosporin and EPO in past. Over summer was taken off
> meds
> as hemoglobin was too high.  He became anemic again and is getting
> blood
> transfusions every 3 weeks despite back on CSA and EPO.   Pt on IVIG
> for
> 2 years.  Pt also with daily fevers for 2 months.  He has enlarged
> liver
> and spleen.  Seen by multiple hematologists, rheumatologists, and
> immunologists with no diagnosis. History of purpuric rash on lower
ext
> 2
> years ago, bx consistent with vasculitis.
>
> Labs: WBC 7, Hgb after pRBC 10, Plt 229, PMN 33 LYM 52, Mono 12, low
> retic count
>
> Albumin 3.6 - low normal, CH 50 - high, C3 and C4 normal, UA
> normal
>
> LFT's mildly elevated twice normal but normal alk phos
>
> IgA 29, IgG 393 on IVIG, IgM 13
>
> Flow Studies
> ABS B lymph count 25 (80-500)
> ABS CD8 count 1617 (157-813)
> CD56 3
> CD19 1
> CD3 96
> CD4 22
> CD8 72
> ABS CD4 487
> ABS T lymph 2156
> ABS CD56 72 (140-440)
> CD4:CD8 0.3
>
> Mitogens pending
> EBV PCR pending
>
> (I am assuming in past vaccine titers prior to IVIG and parvovirus
were
> looked for, but currently I don't have that info)
>
> Bone Marrow BX
> - Red cell hypoplasia, marked.
> - Hypercellular marrow (approximately 90% cellularity) with:
> - Predominance of myeloid precursors with complete maturation.
>  - Scant numbers of erythroid precursors with incomplete maturation.
> - M:E ratio is estimated at greater than 20:1.
> - Megakaryocytes appear increased in number.
> - No lymphoma, granulomatous inflammation or metastatic tumor
> identified
>
> I saw a case of XLP in the literature similiar to this with red cell
> aplasia, decreased CD4:CD8 ratio, hypogam, and undetectable B cells
in
> circulation.  Can you give me your opinion?
>
> Thank you,
>
> Paul Ogershok MD
> pogershok at hsc.wvu.edu 
> Fellow Allergy/Immunology
> West Virginia Univ. School of Medicine
>
>
>
Kathleen E. Sullivan MD PhD
Associate Professor of Pediatrics
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 215-590-3044