[CIS PIDD] [cis-pidd] Laryngeal papilomatosis in boy with CGD awaiting BMT

[CIS-PIDD]

Thanks Ricardo. Very useful information and I would love a copy of this paper. I have a colleague here who is fluent in Spanish so should be able to help us interpret it. We should certainly consider trying this as he needs a BMT and this may help clear the papillomatosis to allow us to proceed to transplant. 
Kind regards
Jane

Associate Professor Jane Peake
Paediatric Immunologist and Allergist
Discipline of Paediatrics & Child Health
School of Medicine | The University of Queensland
Lady Cilento Children's Hospital| 501 Stanley Street | South Brisbane QLD 4101
j.peake at uq.edu.au



________________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Sunday, 12 March 2017 10:22 AM
To: CIS-PIDD
Cc: 'jaime inostroza'
Subject: RE:[cis-pidd] Laryngeal papilomatosis in boy with CGD awaiting BMT

Dear Jane

I am writing on behalf of Dr. Jaime Inostroza,  director of the Jeffrey Modell Center for Diagnosis  and Research in primary immunodeficiencies in Temuco, Chile. I mentioned your question to him because I was aware of an upcoming publication by his team.

They evaluated  a 7-yer-old boy with chronic recurrent laryngeal papilomatosis requiring multiple surgeries since infancy. No immunodeficiency was identified. A biopsy of his lesions confirmed the presence of HPV serotype 6. Based on several publications about the use of the tetravalent HPV vaccine at 9 years of age this boy received 3 doses of Gardasil. The second and third doses were given at 6 mo intervals. His papilomatosis has cleared completely for the first time in his life. He leads now a normal life and has remained free of recurrences  for over 1 year. This patient case will be published now in the Chilean Journal of Pediatrics. I am requesting the English abstract and references in case you are interested.

CGD patients have normal antibody and cell mediated immunity and usually have normal responses to vaccines. You could consider the therapeutic use of HPV for your patient.

Ricardo Sorensen, M.D.
Clinical Professor of Pediatrics
LDU Health Science Center and Children’s Hospital, New Orleans
Head, Jeffrey Modell Center for Diagnosis and Research
in New Orleans
Honorary Professor, Universidad de la Frontera. Temuco, Chile

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Saturday, March 11, 2017 5:39 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] Laryngeal papilomatosis in boy with CGD awaiting BMT

*EXTERNAL EMAIL: EVALUATE*


Dr. Peake:

We've had some decent experience with nebulized cidofovir.   See Giles et al., Ped Resp Rev 2006.

The more curious question is why an XL-CGD patient would have -- assuming it's confirmed as HPV positive -- a recurrent viral infection.

Good luck with this case.

   - K

Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
Section of Infectious Diseases  |  Department of Pediatrics  |  Comer Children's Hospital  |  University of Chicago
5841 S Maryland Ave,  MC 6054,  Chicago  IL  60637 Office phone: 773-702-9281  |  Pager: 773-702-6800 x1744  |  Fax: 773-702-1196

________________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, March 09, 2017 5:40 AM
To: CIS-PIDD
Subject: [cis-pidd] Laryngeal papilomatosis in boy with CGD awaiting BMT

​​Dear all
I was wondering if anyone could help with a patient we have?

We have a 13yo boy with X-linked CGD (previous liver abscess, pulmonary aspergillosis, chronic lung disease) who we were working up for BMT. He has had some palatal ulcers (biopsy - granuloma; extensive microbiological workup revealed no organism) and multiple lymph nodes in his neck for a number of months.  He was admitted prior to transplant to improve nutrition and to investigate some potential foci of infection. In the workup for transplant he was noted to have on MRI asymmetric but circumferential thickening of the soft palate, uvula, palatine tonsils, oropharyngeal mucosal space, epiglottis, aryepiglottic folds, larynx and larynopharynx. PET scan showed diffuse moderate to intense FDG uptake in the same areas. On initial bronchoscopy he was noted to have some supraglottic nodules which were presumed to be granulomas and were not biopsied. A course of steroids were commenced with prednisone 1mg/kg given for 10 days then weaned over the following 3 weeks. He then started to get increasing problems with his upper airways with production of a lot of purulent mucus (no growth) and some difficulty swallowing. On repeat  laryngoscopy he was found to have diffuse severe papillomatosis of the whole of the larynx with copious overlying purulent exudate. Histology would be consistent with papillomatosis and is p16 positive and we are awaiting PCR for HPV. He responded well initially to debridement and relatively high dose dexamethasone (he already was covered with broad spectrum antibiotics and ambisome). Due to a rapid return of his supraglottic and glottic swelling which followed weaning of his steroids and cessation of antibiotics (no bacterial growth), he is to have further debridement tomorrow just 2 weeks after this was first undertaken.

Has anyone encountered HPV this in this setting? If so, what treatments have you tried? What about BMT with  laryngeal papillomatosis ?

Any help or suggestions greatly appreciated

Kind regards

Jane

Associate Professor Jane Peake
Paediatric Immunologist and Allergist
Discipline of Paediatrics & Child Health School of Medicine | The University of Queensland Lady Cilento Children's Hospital| 501 Stanley Street | South Brisbane QLD 4101


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