[CIS PIDD] a patient with GCD+lymphoma

[Saul Oswaldo Lugo Reyes]

   Dear Dr. Kim,

   We cannot conclude cancer is frequent in CGD, but we are aware of
at least three other cases of lymphoma, as well as leukemia and some
solid tumors reported before (please find an attached pdf)

    If histology is conclusive, there's a good chance it is lymphoma.

   I'm sorry I cannot help with the resistant infections, it is a bit
of a problem. Aspergillus might not resolve until after stem cell
transplantation.

     Good luck.  A very interesting case.

 Sincerely,

  Saul.


2012/9/21 YaeJean Kim <yaejeankim at skku.edu>:

>

>

> 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

>

>

> The CIS-PIDD listserv is supported by:

> Clinical Immunology Society - The science & practice of human immunology

>

> P: +1.414.224.8095

> E: info at clinimmsoc.org

>

> Not a member of CIS? Please visit www.clinimmsoc.org to join!




-- 
Saúl Oswaldo Lugo Reyes

Investigador en Ciencias Médicas
Unidad de Investigacion en Inmunodeficiencias
9° piso, Torre de Investigacion,
Instituto Nacional de Pediatria
Coyoacan 04530 DF, Mexico
+52(55)10840900 ext. 1866
--------------------------------------
"La penicilline guérit les humains; le vin les rend heureux"
"If penicillin cures sicknesses, Jerez resuscitates dead people". -Sir
Alexander Fleming
-------------- next part --------------
A non-text attachment was scrubbed...
Name: Lugo Reyes JACI2011.pdf
Type: application/pdf
Size: 74899 bytes
Desc: not available
Url : <http://seven.pairlist.net/pipermail/pagid/attachments/20120921/8e0d2ac5/attachment-0001.pdf>