Table 6.

Summary of Models and Histopathologic Features Present

Mouse Strain	Human Cells	Mast Cell Hyperplasia	Eosinophil Hyperplasia	Histiocytosis with or without hemophagocytosis	Secondary HLH/MAS-Like Disease	Secondary HLH/MAS-Like Disease with CNS Involvement
NSG	Nonea	-	-	-	-	-
NSG	Humanized	-	-	+, subset	-	-
	(hCD34+ HSC)b
NSG	Transduced	-	-	+, subset	-	-
	hCD34+ HSCc
NSG	Leukemia PDXc	-	-	+, subset	-	-
NSG-SGM3	Nonec	+, Md	-	-	-	-
NSG-SGM3	Humanized	+, M and He	+, H	-	+, subset	+, subset
	(hCD34+ HSC)c
NSG-SGM3	Transduced	+, M and H	+, H	-	+, subset	-
	hCD34+ HSCc
NSG-SGM3 or NRG-SGM3	Leukemia PDXc	+, M and H	+, H	-	+, subset	-

Abbreviations: HLH/MAS, hemophagocytic lymphohistiocytosis/ macrophage activation syndrome; CNS, central nervous system; HSC, hematopoietic stem cell; PDX, patient derived xenograft

^aHistorical controls

^bAs reported in reference 39

^cDescribed in current manuscript

^dM, murine cells

^eH, human cells