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

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Definition

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%)                                                            
                                                                           
Other relevant tests
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Page last modified 21:50, 3 Mar 2008 by Chris Concannon