Summary of the Major Types of Nonnasal CD56+ Lymphomas, Based on Information From Current Series and Cases Reported in the Literature
| . | Nasal-Type NK/T-Cell Lymphoma . | Aggressive NK Cell Leukemia/Lymphoma . | Blastoid NK Cell Lymphoma . | Other Specific Lymphoma Types With CD56 Expression . |
|---|---|---|---|---|
| Age | Usually adults | Usually young or middle-aged adults | Adults | Variable |
| Sex | M > F | M ≥ F | ? | Variable |
| Clinical presentation | Presenting with extranodal disease, often involving multiple sites (high stage disease at presentation). Skin, upper aerodigestive tract, soft tissues, testis and gastrointestinal tract are commonest sites of involvement. | Presenting with fever and systemic symptoms. Often have hepatosplenomegaly and sometimes lymphadenopathy. | Presenting with extranodal disease, especially skin | Variable; either nodal or extranodal presentation. Distinctive features may be seen in some specific lymphoma types, such as hepatosplenic γδ T-cell lymphomas. |
| Histologic features | Lymphoma cells often show irregular nuclear foldings and granular chromatin. They can be small, medium-sized or large; either a single cell type predominates, or a mixture of cell types. May be variably admixed with inflammatory cells. Necrosis common. Angiocentric growth may be identified. | Diffuse, monotonous infiltrate of medium-sized cells with condensed chromatin. Nuclei often appear round. May show angiocentric growth and necrosis. | Diffuse, monotonous infiltrate of medium-sized cells with fine chromatin and frequent mitoses. Resembling lymphoblastic lymphoma or granulocytic sarcoma. | Variable, corresponding to the specific lymphoma types. |
| Commonest immunophenotype | CD2+, CD3/Leu4−, CD3ε+, CD56+, CD16−, CD57− | CD2+, CD3/Leu4−, CD3ε+, CD56+, CD16−/+, CD57− | CD2−/+, CD3/Leu4−, CD3ε+, CD56+, TdT− | CD2+, CD3/Leu4+, CD3ε+, CD56+, other T-lineage markers+ |
| T-cell receptor genes | Germline | Germline | Germline | Rearranged |
| Association with EBV | >90% | >90% | 0% | Variable, usually negative |
| Clinical behavior | Aggressive, with early dissemination. Relapse is very common despite initial response to chemotherapy. | Fulminant course | Aggressive | Aggressive |
| . | Nasal-Type NK/T-Cell Lymphoma . | Aggressive NK Cell Leukemia/Lymphoma . | Blastoid NK Cell Lymphoma . | Other Specific Lymphoma Types With CD56 Expression . |
|---|---|---|---|---|
| Age | Usually adults | Usually young or middle-aged adults | Adults | Variable |
| Sex | M > F | M ≥ F | ? | Variable |
| Clinical presentation | Presenting with extranodal disease, often involving multiple sites (high stage disease at presentation). Skin, upper aerodigestive tract, soft tissues, testis and gastrointestinal tract are commonest sites of involvement. | Presenting with fever and systemic symptoms. Often have hepatosplenomegaly and sometimes lymphadenopathy. | Presenting with extranodal disease, especially skin | Variable; either nodal or extranodal presentation. Distinctive features may be seen in some specific lymphoma types, such as hepatosplenic γδ T-cell lymphomas. |
| Histologic features | Lymphoma cells often show irregular nuclear foldings and granular chromatin. They can be small, medium-sized or large; either a single cell type predominates, or a mixture of cell types. May be variably admixed with inflammatory cells. Necrosis common. Angiocentric growth may be identified. | Diffuse, monotonous infiltrate of medium-sized cells with condensed chromatin. Nuclei often appear round. May show angiocentric growth and necrosis. | Diffuse, monotonous infiltrate of medium-sized cells with fine chromatin and frequent mitoses. Resembling lymphoblastic lymphoma or granulocytic sarcoma. | Variable, corresponding to the specific lymphoma types. |
| Commonest immunophenotype | CD2+, CD3/Leu4−, CD3ε+, CD56+, CD16−, CD57− | CD2+, CD3/Leu4−, CD3ε+, CD56+, CD16−/+, CD57− | CD2−/+, CD3/Leu4−, CD3ε+, CD56+, TdT− | CD2+, CD3/Leu4+, CD3ε+, CD56+, other T-lineage markers+ |
| T-cell receptor genes | Germline | Germline | Germline | Rearranged |
| Association with EBV | >90% | >90% | 0% | Variable, usually negative |
| Clinical behavior | Aggressive, with early dissemination. Relapse is very common despite initial response to chemotherapy. | Fulminant course | Aggressive | Aggressive |