[CIS PIDD] a patient with GCD+lymphoma

[YaeJean Kim]

Dear all,

I have a very complicated patient who has AR-CGD and lymphoma and would
like to ask your opinion about.
I apoplogize but this is lengthy.

This is a 13 year old boy who has been previously healthy without any
significant underlying illness (no family hx, no consanguineous
parents) until mild July this year when he developed fever and right ear
pain.

He was initally managed for *AOM* but it progessed and *mastoiditis* was
suspected.
He developed *right facial nerve palsy* and also developed pain in the
right hip joint for which* osteomyelitis* of the right pelvic bones was
suspected. Brain abscess was also found. He had some lymphadenopathy but LN
biopy was non-diagnostic. Of note, his *ear discharge grew Aspergiollus.*

*He was transferred to us on 8/20/2012 after he spent 6 weeks in other
centers.*
I susupected for AR-CGD first and started to evaluate further and managed
for suspected serious invasive infections (bacterial+fungal)
Additional culture studies was non-diagnostic except *ear discharge again
grew Aspergillus. *
At our hopspital the result of evaluation was following.

*brain abscess*
*AOM, **mastoiditis, facial nerve palsy (Rt)*
*massive Rt internal jugluar vein thrombus (septic?)-Lemierre syndrome?*
*endocartitis (vegetation on TV valve?)*
*osteomyelitis of multiple pelvic bones (Rt)*

I have given him cefepime, vancomycin, penicillin, ambisome (-> now on
voriconzaole).

He continued to have fever for 6 weeks but at least vegetation like lesion
in the echo diappeared and his pelvic bone pain improved...
So I thought his condition was slowly improving...but then he developed
mental changes with brain lesion became bigger...
An emergent operation was done to remove the brain mass last week (it was
not a frank abscess). At that time, he also received granulocyte infusions
twice and steroid (for antiinflammatory + brain edema..)

*It was reported that brain tissue showed lymphoma lesions, yesterday*. Per
our pathology person who is an expert on lymphoma. it is staining pattern
is typical for lymphoma cancer cells (immune stains are positive also) and
I also saw the slides myself!!
The *GCD gene test was reported that he has muation in NCF1 gene, today.*

So, this patient has lymphoma and CGD. We did bone marrow and will do
pet-CT soon to evaluate the extend of lymphoma...
but I am concern which spot is lymphoma and which spot is infection....

1. My first question,  is it  a common to see cancer in CGD patient?

2. Anybody has similar experience in a patient with CGD and unusual
location of lymphoma?
This could be not lymphoma but something else related to severe
inflammation or infection? (cannot explain for immunostain, then)

3. And, what should I do at this point?

When to start lymphoma chemo? our HO people are on-board for chemo and
wants to start chemo soon but I am very worried...because I don't think his
infection is not controlled, yet...but the tumor starts to grwo faster, we
have to do something anyway..
Should I give more neutrophil infusions before they give chemo? or when
they give anticance tx (chemo or RT)?
The prednisone (1 mg/kg/day), should I stop it or continue?

For infection, I think aspergillosis is the main thing..but I cannot rule
out other bacterial process since he has been on antibiotics already for
several weeks before he came to me...I just can't stop any of them...would
you stop any of those Abt (cefepime, vancomycin, penicillin, voriconzaole)?
Septic emboli vs. tumor emboli?

Again, I apologize for this lengthy mail but I am very concerned about this
boy's condition and hope to hear others' opinion or experiences.
I appreciate any advice or insights from you.

Regards,


YaeJean

---------------------------------------
Yae-Jean Kim, MD
Assistant Professor
Division of Infectious Diseases
Department of Pediatrics
Sungkyunkwan University School of Medicine
Samsung Medical Center
50 Irwon-dong Gangnam-gu
Seoul, Korea
Tel)  +82-2-3410-0987  Fax) +82-2-3410-0043
yaejeankim at skku.edu
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