Table 3. Patients with a diagnosis of... | American Society of Hematology

Table 3.

Patients with a diagnosis of HLH-related disorders

Patient	Disease	Age/sex	NK LU	% NK	% perforin
Patient	Disease	Age/sex	NK LU	% NK	NK	CD8	CD56
P15	Chediak-Higashi syndrome	7 mo/M	0.0↓	3	95	1	34
P16	XLP	15 mo/M	1.5↓	<1↓	99	32↑	23
P17	JRA + MAS	22 y/F	4.6	5↓	82↓	2↓	7↓
Normal values
		0-1 y		3-17	91-97	0-3	4-30
		1-15 y		4-22	81-91	2-11	12-34
			>3.2
		15-50 y		7-31	86-98	8-28	30-77

Patient	Disease	Age/sex	NK LU	% NK	% perforin
Patient	Disease	Age/sex	NK LU	% NK	NK	CD8	CD56
P15	Chediak-Higashi syndrome	7 mo/M	0.0↓	3	95	1	34
P16	XLP	15 mo/M	1.5↓	<1↓	99	32↑	23
P17	JRA + MAS	22 y/F	4.6	5↓	82↓	2↓	7↓
Normal values
		0-1 y		3-17	91-97	0-3	4-30
		1-15 y		4-22	81-91	2-11	12-34
			>3.2
		15-50 y		7-31	86-98	8-28	30-77

A patient with Chediak-Higashi syndrome in accelerated phase showed values within the normal range for his age. A patient with XLP showed markedly increased perforin-expressing CD8⁺ T cells. A patient with JRA complicating MAS showed decreased proportions of perforin-expressing cells in all cytotoxic cell types.

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