[CIS PIDD] [cis-pidd] HIES and lung transplantation
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Tue Mar 1 06:48:37 EST 2016
We are still trying to sort out the vasculopathy. Probably some form of rejection. It isn't a common complication and we don't know whether the HIES played a role-with the interactions between the STAT wildtype lung and pulmonary vessels and her STAT deficient WBCs. Hard to sort out with one patient but ill let you know if we do.
Alexandra
Alexandra Freeman MD
Staff Clinician
LCID/NIAID/NIH
On Mar 1, 2016, at 1:15 AM, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Thanks Alexandra – very useful to know how your patient fared. Sounds like she was more compromised from a respiratory POV prior to transplant than ours and the difficult surgery may have contributed to her outcomes. Was the vasculopathy thought to be a medication issue (eg calcineurin inhibitor) or graft rejection? Will keep you informed if we go ahead.
Regards,
Dr Ben McGettigan FRACP FRCPA
Clinical Immunologist
Address: Immunology Dept, Fiona Stanley Hosptial, L1 Pathology, Locked Bag 100
Palmyra DC, Western Australia 6961
Fiona Stanley Hospital Tues and Friday all day:
Ph (08) 6152 8303, Fax (08) 6152 8051
PathWest QEII Thurs all day:
Ph (08) 6383 4303, Fax (08) 9346 3160
Email: Benjamin.McGettigan at health.wa.gov.au<mailto:Benjamin.McGettigan at health.wa.gov.au>
Website: www.health.wa.gov.au<http://www.health.wa.gov.au>
Delivering a Healthy WA
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, 1 March 2016 10:54
To: CIS-PIDD
Subject: RE:[cis-pidd] HIES and lung transplantation
Hi, Here is an update to our NIH patient that had a double lung transplant. She is the only patient we follow with AD-HIES who has had a lung transplant.
She had severe lung disease by her 20s with large pneumatoceles, one prior lobectomy, and chronic infection with Mycobacteria abscesses, Aspergillus and Stenotrophomonas. She had sepsis related to a line infection during her treatment with M. abscesses that led to ARDS and had further compromised her lung function and so at the time of transplant, she was very limited and required BIPAP most of the time (and had been oxygen dependent for a few years). The M. abscessus was hard to get under control and she was rejected at a few centers for that, but finally accepted when she was AFB smear negative. She was in her late 20s at the time of transplant.
Transplant course was very hard- they had lots of trouble getting her lungs out, lots of bleeding, required cardiopulmonary bypass and was a very long surgery- and then the new lungs were too big and they had to trim them a few days later to close her chest. This led to multi-organ failure, but she rallied and eventually became stable and able to be discharged. But, the Aspergillus had spilled during the rough surgery and this led to several problems down the line- and the Aspergillus was resistant to posaconazole and she was intolerant of voriconazole. So she popped up with abscesses of Aspergillus on her diaphragm and endobronchially- that we cleared with liposomal amphotericin- but a couple years out she developed a mediastinal mass of the same Aspergillus that obliterated her right pulmonary artery. We were able to treat the mold (liposomal ampho and then isavuconazole), but the artery was fibrosed and never opened up and this led to a big decline with pulmonary hypertension and then there was associated transplant vasculopathy and eventually she died about 3 years from transplant from pulmonary hemorrhage.
Post-Transplant she was on IVIG, bactrim as well as suppressive M. abscessus abx for one year and an anti fungal. She never had trouble with bacterial infections and didn't develop bronchiectasis or pneumatoceles. The main infection issue was the Aspergillus, which was more because of the spilling during the difficult surgery than related to her underlying HIES. She did have the polyomavirus as you mentioned- WU in her lungs (asymptomatic) and BK in her urine/blood- also asymptomatic. She was not on much immune suppression, due to the mold, didn't have much trouble with classic lung rejection- but did have this funny vasculopathy (beyond the infected pulmonary artery).
She did have a few good years, was off oxygen, able to take care of her son, so we were all glad she did do the transplant, although we wished she had survived much longer.
Happy to answer any other questions about her course. We had initially planned to follow this with a marrow from the lung donor, but she had become too sick and unstable to wait for a good match for both the lung and bone marrow- and the marrow harvest was very poor.
Hope that helps some!
Alexandra
—
Alexandra Freeman MD
Laboratory of Clinical Infectious Diseases
NIAID, NIH
NIH, Bldg 10,Room 12C103
9000 Rockville Pike
Bethesda, MD 20892
Phone 301-594-9045
Fax 301-496-0773
freemaal at mail.nih.gov<mailto:freemaal at mail.nih.gov>
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From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Monday, February 29, 2016 9:35 PM
To: CIS-PIDD
Subject: [cis-pidd] HIES and lung transplantation
Hi all,
Thanks in advance for your collective expertise regarding a recent referral:
55F with AD hyper IgE syndrome (known STAT 3 L706M mutation) referred by Respiratory to help assess suitability for lung transplantation. She has previously been managed by a colleague.
• HIES diagnosed in the '80s, recurrent childhood infections (predominantly cutaneous early on)
o Previous recurrent skin furunculosis and mycobacterium terrae tenosynovitis
o Eczema reasonably well controlled (more prominent as a child)
o Blind in R eye secondary to ?polymicrobial infection
o A trial of treatment with IVIg in the late '90s provided no symptomatic benefit and was ceased
o Never had interferon gamma therapy
• Previous mucinous tumour of the ovary
o TAH and BSO 10 years ago, no recurrence since
• L ear high frequency hearing loss - aminoglycoside related.
• Recently lung infections have become most prominent, with progressive cystic/saccular bronchiectasis.
o Colonized with pseudomonas aeruginosa (last sputum 22/02/16 – abundant growth pseudomonas aeruginosa -sens to tazocin, ciprofloxacin, resistant to gentamicin) and Aspergillus fumigatus in past
• No evidence of fungal or opportunistic infection on CT chest
o Recurrent infective exacerbations about 2x per year on average.
o On prophylactic azithromycin; tobramycin nebs 80mg BD - for years.
o Severe obstruction on recent PFTS
• Seretide 250/25 2 puffs BD and Salbutamol MDI
o Oxygen dependant since Jan 16.
o Exercise tolerance dropped from 1-2km in late 2015 to just around house since early Jan this year (minimal improvement in ex tolerance despite multiple courses of broad spectrum antibiotics)
o Previous R sided empyema in Jan 15 - had pleural drainage with ICC and received intra-pleura thrombolytic therapy (tPA-DNAse); no VATS decortication/thoracotomy.
• Reports repeated and problematic skin infections / boils with previous courses of steroids (last received approx 20 years ago) and we understand this was considered the main factor precluding consideration of a lung transplant in the past.
• Other background:
o Ex-smoker (smoked 4-5 cigs/day during teenage years; ceased about 40 years ago)
o ETOH - rarely drinks
o Medically retired; used to work in finances (in police station) for 35yrs
o Previously worked on family's farm, exposed to herbicides.
o Hobbies: wood works (not wearing mask usually)
o Pet - dog; no birds
o Travel - Europe last year; Bali 5 yrs ago
o Married - two grown up kids (daughter - has hyper IgE syndrome as well)
o No previous fractures/
She has been reviewed by an ID physician who has stated “there are no infectious disease contraindications to transplantation” but clearly she is high risk for complications. I have only been able to find one paper describing a patient with HIES having a lung transplant in the absence of HSCT (NIH group describing WU polyomavirus in Respiratory epithelial cells post transplant).
I would be grateful to hear from others regarding their experience with lung transplant in HIES and/or opinions/suggestions on the above case.
Kind Regards,
Ben
Dr Ben McGettigan FRACP FRCPA
Clinical Immunologist
Address: Immunology Dept, Fiona Stanley Hosptial, L1 Pathology, Locked Bag 100
Palmyra DC, Western Australia 6961
Email: Benjamin.McGettigan at health.wa.gov.au<mailto:Benjamin.McGettigan at health.wa.gov.au>
Website: www.health.wa.gov.au<http://www.health.wa.gov.au>
Delivering a Healthy WA
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