[PAGID] Thymoma, pan-hypogammaglobulinemia, T cell deficiency (# and quality) and CMV encephalitis/r

[Saxon, Andy M.D.]

Thanks!

Do you think the MDS was in fact pure red aplasia? NO, most likely was
medication/secondary to disease.

Has CMV been found in the thymoma? Not known. Done years ago at outside
institution. Very unlikely they looked for it.

HLH - currently BM and others show no evidence for this.

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jack Bleesing
Sent: Thursday, October 09, 2008 1:42 PM
To: pagid at list.clinimmsoc.org
Cc: Viera, Loida M.D.; Yang, Otto
Subject: Re: [PAGID] Thymoma, pan-hypogammaglobulinemia,T cell
deficiency (# and quality) and CMV encephalitis/r

Do you think the MDS was in fact pure red aplasia? Has CMV been found in
the thymoma?

Other suggestions:

For CMV issues:

- cytogam (up to 2x per week)
- cidofovir (3 times per week with probenecid). This has worked in
several of our patients, who did not clear with Foscarnet (and certainly
not ganciclovir)
- leflunomide in combination with foscarnet.

Work her up for a HLH-like disorder, causing fevers, cytopenias, etc.
etc (CMV or another virus could be trigger). This would be helpful, as
IL-2 could provide fuel to the HLH-fire. HLH can complicate any
immunodeficiency with the types of defect you describe.Other cytokines,
such as INF-G would a have similar concern. If no evidence of HLH, would
think that low-dose IL-2 is worth a shot.

JB


------------------------------------------------------------------------
---
Jack J.H. Bleesing, M.D., Ph.D.
Associate Professor of Pediatrics
Cincinnati Children's Hospital Medical Center Division of
Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> "Saxon, Andy M.D." <ASaxon at mednet.ucla.edu> 10/9/2008 1:48:13 PM >>>

Thymoma, pan-hypogammaglobulinemia, T cell deficiency (# and quality)
and CMV encephalitis/retinitis.  Use of IL-2???

See below.
1. Has anyone tried IL-2 in this setting?  We have been asked to
consider trying it.
2. What do you think about trying "low dose" IL-2 3. Any other
suggestions?

Andy Saxon
UCLA

This patient is a woman who is approximately 50 years old. She was
previously healthy with no significant medical problems (other than
prophylaxis for a positive PPD and thymectomy for a benign thymoma)
until 2001, when she was found to have Pneumocystis pneumonia. HIV
testing was negative. Thereafter, she also developed CMV retinitis of
the left eye in 2004, with a recurrent episode 2005. Thereafter she was
maintained on bactrim and oral ganciclovir prophylaxis. In 2004 she
developed pancytopenia requiring chronic RBC transfusions and g-CSF. In
2007 she received dacogen for possible myelodysplastic syndrome, with
some reduction in RBC transfusion requirements.

In late July of this year, she developed worsening confusion, and was
found to have CMV encephalitis with scattered brain lesions, CSF PCR
positive, and blood PCR positive. She also had reactivation of CMV
retinitis in the left eye and new CMV retinitis of the right eye,
requiring intravitreal injections of foscarnet. She was treated with IV
foscarnet, with clearance of CMV PCR tests of blood and CSF. Multiple
HIV tests have been negative over several years. In addition, PCR was
negative (both viral load and genotyping tests) and special detection
tests for unusual strains of HIV done at the CDC were negative. She was
initially neutropenic and had a CD4 T cell count around 100 while on
ganciclovir, but upon stopping ganciclovir, her neutropenia resolved and
her CD4 T cell count has been stable around 200 (CD8 count around 500).
Her T cell studies have shown a lack of mature T cells.  We assume that
ganciclovir and CMV infection both contributed to these depressed
counts. Multiple flow cytometry tests have demonstrated low NK cells and
nearly absent B cells. She has pan-hypogammaglobulinemia, which has been
treated with IV Ig. Overall, the working diagnosis is Good Syndrome.
Currently she remains on maintenance foscarnet. Her only acute issue is
occasional febrile episodes, without a clear source (she has had scans
of the abdomen and chest, and numerous cultures of blood), and ongoing
CMV retinitis.

________________________________
IMPORTANT WARNING: This email (and any attachments) is only intended for
the use of the person or entity to which it is addressed, and may
contain information that is privileged and confidential. You, the
recipient, are obligated to maintain it in a safe, secure and
confidential manner. Unauthorized redisclosure or failure to maintain
confidentiality may subject you to federal and state penalties. If you
are not the intended recipient, please immediately notify us by return
email, and delete this message from your computer.

IMPORTANT WARNING:  This email (and any attachments) is only intended for the use of the person or entity to which it is addressed, and may contain information that is privileged and confidential.  You, the recipient, are obligated to maintain it in a safe, secure and confidential manner.  Unauthorized redisclosure or failure to maintain confidentiality may subject you to federal and state penalties. If you are not the intended recipient, please immediately notify us by return email, and delete this message from your computer.