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

[CIS-PIDD]

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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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