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

[Jack Bleesing]

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


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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.

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