Lymphoblastic Lymphoma Expressing Natural Killer Cell Phenotype with Involvement of the Mediastinum and Nasal Cavity

Abstract

Most CD56+ lymphomas display polymorphic and angiocentric/angiodestructive histologic features and are closely related to Epstein-Barr virus (EBV) infection. We report a 47-year-old Japanese man with CD56+ lymphoma that showed histologic features of lymphoblastic lymphoma with mediastinal and nasal involvement and an aggressive course. A sample specimen showed the histology of lymphoblastic lymphoma with a positive reaction for terminal deoxynucleotidyl transferase (TdT) but no angiocentric/angiodestructive features. Transmission electron microscopy revealed a few membrane-bound electron-dense granules in their cytoplasm. Immunohistochemically, lymphoma cells exhibited CD56+ cytoplasmic CD3 (cCD3)+ TdT+. A Southern blot analysis showed no integration of EBV and human T-lymphotrophic virus 1 (HTLV-1) and no rearrangement of the T-cell receptors or immunoglobulin heavy chain genes. This unusual lymphoblastic lymphoma exhibiting cCD3 + CD56 + TdT + TCR- is assumed as an immature or progenitor natural killer cell lineage.

Complete remission in advanced blastic NK‐cell lymphoma/leukemia in elderly patients using the hyper‐CVAD regimen

“…Furthermore, one of these seven was suffering from pulmonary aspergillosis [17], while another patient's follow-up was limited to 5 months [15]. Twenty-three of the 47 patients who succumbed to their disease failed to attain CR (and hence never experienced disease-free survival) [9,10,[13][14][15]17,[25][26][27][28][29]. Twenty-three others experienced an average disease-free survival of 8.3 months before succumbing to their disease [6,9,14,15,21,30].…”

Clinicopathologic and genotypic study of extranodal nasal-type natural killer/T-cell lymphoma and natural killer precursor lymphoma among Koreans

“…34 Of note, those NK precursor tumors with or without myeloid marker express TdT detected by immunohistochemical method. [11][12][13] Because there has been no information on the TdT expression in the normal immature NK precursor, TdT expression in blastoid NK-cell lymphoma raised a debate on their cellular nature and diagnostic terminology, either lymphoblastic NK-cell lymphoma or blastoid NK-cell lymphoma. Although TdT initially was considered as a marker of immature lymphoid cells, many studies showed TdT expression in overall 18% of acute myeloblastic leukemia, and TdT positivity is more common in the immature cell types (M0, M1).…”

Nasal CD56 positive small round cell tumors

“…Case 2 presented with swelling of the lymph nodes of the neck. Case 1 has been previously reported [16] and also presented as a mediastinal tumor. Tumor cells were found in bone marrow in three cases (cases 1, 3, and 9).…”

“…Recently, NK phenotype of lymphoblastic lymphoma or blastic NK-cell lymphoma has been described [6,10,12,13,15,16,22,28]. Patients are predominantly middle-aged to elderly.…”