Table 5.

Summary of published cases of HSCT in STAT3-HIES, with original series, transplant characteristics (age at HSCT, donor type, conditioning regimen, lymphoma as indication), presence and type of GvHD, donor chimerism at latest evaluation, and follow-up data with organ-specific status post-transplant

Series	Sex	Age at HSCT	Donor	Conditioning	Lymphoma	GvHD	Donor chimerism	Organ status post-HSCT			Survival
								Skin	Lung	Other
Nester et al. [115]	M	46	MSD	MAC	Y	Acute	Not known	–	Interstitial pneumonitis leading to death	–	N
Goussetis et al. [118]	M	15	MSD	MAC	Y	–	Full	–	Infection-free	–	Y
	F	16	MSD	MAC	Y	–	Full	–	Infection-free	–	Y
Patel et al. [135]*	F	14	Haploidentical	RIC	–	–	Full	Abscess formation once	Infection-free	–	Y
Yanagimachi et al. [136]	F	8	MUD	RIC	–	Acute	Full	–	Recurrent aspergillosis	–	Y
	M	23	MSD	RIC	–	–	Mixed	–	Recurrent pneumatocele	–	Y
Harrison et al. [117]	M	7	MUD	RIC	–	–	Full	–	Requires nocturnal CPAP	Scoliosis, fracture	Y
	F^	7	MUD	MAC	–	Acute	Full	–	Stable appearance on CT, improved PFTs	Scoliosis, HTN	Y
	M	13	MSD	RIC	–	Acute	Full	–	Stable appearance on CT, improved PFTs	Anterior MI	Y
	M	14	MSD	RIC	–	Acute	Full	Dry, no infection	Improved appearance on CT, improved PFTs	Resolved autoimmune neutropenia	Y
	F	17	MUD	RIC	–	Acute	Full	–	Improved appearance on CT	–	Y
	M	18	MUD, MUD	RIC, RIC	–	–	Full*	–	Stable CXR changes	Septic arthritis of hip	Y
	M	13	MUD	RIC	–	–	Full	Dry, no infection	Improved symptoms	–	Y
	F	6	MSD	RIC	–	–	Full	Dry, no infection	Improved symptoms	–	Y

Key: MSD, matched sibling donor; MUD, matched unrelated donor; MAC, myeloablative conditioning; RIC: reduced intensity conditioning; GvHD, graft-versus-host disease; CPAP, continuous positive airway pressure (ventilation); HTN, hypertension; PFTs, pulmonary function tests; MI, myocardial infarct

^This patient was originally reported by Gennery et al. in 2000 [116]

^*This patient had a second transplant following hyperacute rejection on D + 13