Clinical features and prognosis of chronic natural killer cell lymphoproliferative disorders

Figures & data

Table 1. Clinical differential diagnosis of CPLD-NK, T-LGL, ANKL and NK/T lymphoma.

	CPLD-NK	T-LGL	ANKL	NK/T lymphoma
clinical manifestation	Insidious onset, mild symptoms (characterized by abnormal blood cells)	Insidious onset, mild symptoms (characterized by abnormal blood cells)	Acute presentation, severe systemic symptoms, combined with hemophagocytic syndrome	Acute manifestations, severe systemic symptoms, accompanied by solid lesions, combined with hemophagocytic syndrome
Peripheral blood NK cells and T cells were detected by flow cytometry	The number of abnormal NK cells was > 2× 10^9 /L and lasted for more than 6 months	The number of abnormal T cells was > 2× 10^9 /L and lasted for more than 6 months	Abnormal T lymphocyte ratio increased	Abnormal NK/T lymphocyte ratio increased
Identification of NK and T cell immune phenotypes	CD3-CD16+CD56+ (NK cell immunophenotype)	CD3+CD8+CD16-/+CD56+CD57+ (T cell immunophenotype)	CD3-CD16+CD56+ (NK cell immunophenotype)	CD3+CD16+CD56+ perforin (NK cell and T cell immunophenotypes)
Pathological smear examination	Monoclonal proliferation of large granular cells	Monoclonal proliferation of large granular cells	Monoclonal proliferation of large granular cells	Monoclonal proliferation of NK cell and T cell
Gene mutation and histological identification	KIR restricted expression, STAT3 and TET2 mutations	STAT3, TET2, STAT5 mutations, TCR gene rearrangement	EBER+, EB virus positive, KIR restricted expression	EBER+, EB virus positive
prognosis	good	good	poor	poor

Notes: Chronic NK-cell lymphoproliferative disorder (CLPD-NK), aggressive NK-cell leukemia (ANKL), T-cell large granular lymphocytes (T-LGL), Epstein-Barr virus (EBV), Epstein-Barr virus-encoded RNA (EBER)

Table 2. Basic characteristics of the 18 patients.

CASE	Age	Sex	First symptoms	EBV expression	source of abnormal NK cells	Associated Diseases	Treatment modality	Prognosis	Follow-up duration (months)
1	51	Female	○	-	BM	No	●	Survival	72
2	44	Female	○	-	BM	No	●	Survival	62
3	61	Female	○△	+	BM	HLH	▪	Death	2
4	48	Female	○△	-	BM PB	HBV (+)	●	Survival	53
5	59	Female	○△▽	-	BM	No	▾	Survival	23
6	47	Male	○□	-	BM PB CSF	No	▴	Survival	3.5
7	58	Female	○□	-	BM PB	HBV (+)	▾	Survival	8.5
8	52	Male	○△	-	BM	HLH	▪	Death	7
9	55	Female	○□	-	BM CSF	No	▪★	Survival	7
10	49	Male	○△▽	-	BM	No	●	Survival	9
11	60	Female	○	-	BM	No	▾	Survival	16
12	72	Female	○	-	BM	No	●	Survival	18
13	75	Female	○	-	BM	No	●	Survival	33
14	52	Female	○△✩	-	BM	No	●	Survival	35
15	46	Male	○✩	-	BM	HLH	▪	Death	2
16	52	Female	○△	-	BM	Sjogren’s syndrome	▾	Survival	10
17	59	Male	○	-	BM	No	●	Survival	89
18	55	Male	○	-	BM	Sjogren’s syndrome	●	Survival	24

Notes: ○Anemia, neutropenia, thrombocytopenia □Neurological symptoms ▽Lymph node enlargement ✩Hepatosplenomegaly △Infection ●Observational follow-up ▪Chemotherapy ▾Immune-related treatment ▴Glucocorticoids alone ★Small molecule drugs (selinexor). BM, bone marrow; HLH, Hemophagocytic lymphohistiocytosis; PB, peripheral blood; CSF, cerebrospinal fluid; HBV, hepatitis B virus.

Figure 1. Clinical symptoms of 18 cases of chronic NK-cell lymphoproliferative disorder.

Figure 2. Treatment in 18 cases of patients with chronic NK-cell lymphoproliferative disorder.

Figure 3. Prognosis of 18 patients with chronic NK-cell lymphoproliferative disorder.

Figure 4. Survival curve of 18 patients with chronic NK-cell lymphoproliferative disorder.