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

[Cunningham-Rundles, Charlotte]

We used IL-2 in 4  CVID or ICL cases: 1 mycobacterial infection, 2 for
warts, 1 for post cryptococcal osteo.  --- it helped #1, did not much in one
case of warts and cleared up very bad genital warts another; not much to see
for the osteomyeletiis as he was on anti fungal too.Keep the dose smallish,
250,000/m2 sq  BID ‹ is you start with less and work up side effects are
minimal or non existent.
   I think you have little to loose. But I guess you are using CVM globulin
too? 

On 10/9/08 1:48 PM, "Saxon, Andy M.D." <ASaxon at mednet.ucla.edu> wrote:


> 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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