A clinical study of 21 patients with hemophagocytic syndrome in 295 cases diagnosed with nasal type, extranodal nature killer/T cell lymphoma

Figures & data

Table 1. Clinical characteristics of patients with NK/T-LAHS.

Characteristics	No. (n = 21, %)
Gender
Male	17/21(81.0)
Female	4/21(19.0)
Age(years), median(range)	35(16–65)
Ann Arbor stage
I – II	5/21(23.8)
III – IV	16/21(76.2)
ECOG PS
0–1	9/21(42.9)
2–4	12/21(57.1)
Fever	21/21(100.0)
Hepatosplenomegaly	14/21(66.7)
Lymphadenopathy	13/21(61.9)
BM involvement	8/21(38.1)
Lymphoma status
At onset	19/21(90.5)
At relapse	2/21(9.5)
Triglyceride>3mmol/L	12/21(57.1)
Fibrinogen<1.5 g/L	16/21(76.2)
Ferritin>2000 ng/mL	21/21(100)
Hemocytopenia involving at least 2 lineages	21/21(100)
Hemophagocytosis in bone marrow	10/21(47.6)
LDH>ULN	18/21(85.7)
Liver dysfunction	21/21(100)

ECOG PS the Eastern Cooperative Oncology Group performance status, BM bone marrow, LDH lactate dehydrogenase, ULN upper limit of normal. The criteria for liver dysfunction is jaundice, alanine aminotransferase (ALT) and/or aspartate aminotransferase (AST) >ULN. Hepatits and drug related damage are not included.

Figure 1. Survival curves of 295 patients with Extranodal natural killer/T-cell lymphoma.

Figure 2. Overall survival curves for patients with and without Extranodal nature killer/T cell lymphoma-associated hemophagocytic syndrome.

Figure 3. Receiver Operating Curve (ROC) for determining the optimal cut-off value of lactate dehydrogenase.

Table 2. Risk factors for NK/T-LAHS.

Parameters	p value
Gender	0.814
Age (> 60 years)	0.863
Ann Arbor (III∼IV)	0.292
ECOG PS (≥ 2)	0.433
B symptoms	0.484
Hepatosplenomegaly	0.003
Lymphadenopathy	0.596
BM involvement	0.019
Lymphoma status	0.159
LDH>314IU/L	0.038

ECOG PS the Eastern Cooperative Oncology Group performance status, BM bone marrow, LDH lactate dehydrogenase.

Table 3. Stratification and frequencies of onset for LAHS based on risk group as defined by the new risk index.

Risk group	No. of factors (%)	No. of patients (%)	LAHS onset (%)
Low risk	0	233(79.0%)	2(0.9%)
Intermediate risk	1	43(14.6%)	6(14.0%)
High risk	2-3	19 (6.4%)	13(68.4%)