Table 2 Overview on systemic and CNS... | American Society of Hematology

Table 2

Overview on systemic and CNS reactivations and partial flares

ID	Genetic defect	Days after first HSCT	Overall DC, %	CD3⁺ DC, %	CD56⁺ DC, %	Protein expression	NK cytotoxicity	Trigger	Immune suppression	Second HSCT	Cause of death, if applicable
Reactivations
P009		65	88	22				EBV CMV AdV	Yes	No	HLH
P074	FHL5	71	1					AdV	No	Yes	Alive
P078	FHL2	80	9			Reduced		CMV	No	No	TRM first HSCT: GVHD, TMA
P042	FHL3	92	2					EBV	Yes	No	HLH, PTLD
P043	FHL2	100	86					EBV	Yes	No	Alive
P023	FHL3	172	10						No	Yes	Alive
P052	CHS	188	30						No	Yes	TRM second HSCT: VOD
P028	FHL2	217	5						No	Yes	Alive
P002	FHL5	276	8	10		Normal		Norovirus	No	Yes	Alive
P003	GS2	444	18						No	Yes	Alive
P038	FHL4	2460	13	5	5	Absent			No	Yes	TRM second HSCT: GVHD
Partial flares
P071	FHL5	27	61	0				CMV	No	Yes	Alive
P077	*	151	29	63	20		Reduced		Yes	Yes	TRM second HSCT: septicemia
P079	FHL5	546	28			Reduced	Normal		No	No	Alive
CNS reactivations
P066	FHL3	27	100			Reduced	Normal		Yes	No	Alive
P001	FHL3	136	9						No	No	CNS disease, possibly HLH†
P022	FHL3	750	12	17	41				No	No	CNS disease, possibly HLH†
P025	CHS	1591	7	0					No	Yes	Alive

ID	Genetic defect	Days after first HSCT	Overall DC, %	CD3⁺ DC, %	CD56⁺ DC, %	Protein expression	NK cytotoxicity	Trigger	Immune suppression	Second HSCT	Cause of death, if applicable
Reactivations
P009		65	88	22				EBV CMV AdV	Yes	No	HLH
P074	FHL5	71	1					AdV	No	Yes	Alive
P078	FHL2	80	9			Reduced		CMV	No	No	TRM first HSCT: GVHD, TMA
P042	FHL3	92	2					EBV	Yes	No	HLH, PTLD
P043	FHL2	100	86					EBV	Yes	No	Alive
P023	FHL3	172	10						No	Yes	Alive
P052	CHS	188	30						No	Yes	TRM second HSCT: VOD
P028	FHL2	217	5						No	Yes	Alive
P002	FHL5	276	8	10		Normal		Norovirus	No	Yes	Alive
P003	GS2	444	18						No	Yes	Alive
P038	FHL4	2460	13	5	5	Absent			No	Yes	TRM second HSCT: GVHD
Partial flares
P071	FHL5	27	61	0				CMV	No	Yes	Alive
P077	*	151	29	63	20		Reduced		Yes	Yes	TRM second HSCT: septicemia
P079	FHL5	546	28			Reduced	Normal		No	No	Alive
CNS reactivations
P066	FHL3	27	100			Reduced	Normal		Yes	No	Alive
P001	FHL3	136	9						No	No	CNS disease, possibly HLH†
P022	FHL3	750	12	17	41				No	No	CNS disease, possibly HLH†
P025	CHS	1591	7	0					No	Yes	Alive

The characteristics of systemic reactivations (≥5 HLH criteria fulfilled), partial flares (<5 criteria fulfilled, HLH-directed treatment administered), and isolated CNS reactivations are shown, including overall and lineage-specific donor chimerism and functional assays. Protein expression refers to perforin in FHL2 and CD107 degranulation in FHL3-5. The detailed parameters of the HLH-2004 criteria at each event are shown in supplemental Table 1.

AdV, adenovirus; TMA, transplantation-related microangiopathy; VOD, veno-occlusive disease.

In this patient, a double heterozygous defect was found, 1 each for FHL3 and 5.

†

These patients displayed severe CNS disease. Isolated CNS-HLH was suspected, but could not be fully proven.

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