Mature T-Cell and NK-Cell Neoplasms (general)

T cell lymphoproliferative disorders are a diverse group of lymphoid neoplasms that are a clonal expansion of the mature T- lymphocytes in bone marrow, blood or other tissues. Because natural killer cells (NK) are closely related and share some phenotypic features, these are categorized together.

Epidemiology

T and NK neoplasms are uncommon and make up approximately 12% of the lymphoproliferative group. Incidence varies by geography, and are more prevalent in Asia. This may be related to the HTLV-1 virus which is endemic in Japan.

   

The WHO Classification of Mature T and NK neoplasms

      A. Leukemic or disseminated

T-cell prolymphocytic leukemia (T-PLL)

T-cell granular lymphocytic leukemia (T-LGL)

Aggressive NK leukemia

       Adult T-cell lymphoma/leukemia (ATCL)

B. Extranodal

Extranodal NK/T cell lymphoma (nasal type)

Enteropathy-type T cell lymphoma

Hepatosplenic T cell lymphoma (HTCL)

Subcutaneous panniculitis-like T cell lymphoma

C. Cutaneous

Blastic NK lymphoma

Mycosis fungoides/Sezary syndrome

Primary cutaneous anaplastic large cell lymphoma

D. Nodal

Peripheral T-cell lymphoma, unspecified

Angioimmunoblastic T-cell lymphoma (AILT/AILD)

Anaplastic large cell lymphoma

Immunophenotyping

Below is a table of the immunophenotypic profiles of major categories of mature T-cell neoplasms:       

Antibody	TCL	AILD	ATCL	T-PLL	Anaplastic LCL	HTCL	T-LGL	NK-LGL
CD2+	91	100	100	97	56	82	100	100
CD3+	66	50	75	98	30	100	100	0
CD5+	83	100	100	95	48	18	80	0
CD7+	49	54	0	90	22	82	83	100
CD4+	66	100	100	49	65	0	2	0
CD8+	12	0	0	24	17	9	85	21
CD4/8+	4	0	0	19	5	0	10	0
CD4/8-	20	0	0	8	13	91	3	79
TCR ab+	76	100	75	90	67	9	80	7
TCR gd+	0	0	0	0	0	91	20	0
TCR-	24	0	25	10	33	0	0	93
CD25+	18	rare	100	12	25	0	0	0
CD30+	8	rare	NA	NA	100	NA	NA	NA
CD56+	11	9	0	10	rare	100	32	71
CD57+	rare	0	0	rare	0	0	95	64
ALK+	0	0	NA	NA	66	NA	NA	NA
EMA+	7	NA	NA	NA	78	NA	NA	NA
CD11b+	rare	0	0	0	0	0	24	42
CD11c+	10	0	0	4	0	0	44	11
other	CD10+ (9%)	CD10+ (50%)		CD117+(17%)	CD10+(12%)	CD16+(36%)	CD16+(32%)	CD16+(71%)
other	CD117+(1%)	bcl-6 (30%)