Primary CD56 + nasal-type T/natural killer-cell subcutaneous panniculitic lymphoma: presentation as haemophagocytic syndrome

Abstract

Natural killer (NK) cells are large granular lymphocytes that mediate cytotoxic reactions which are not restricted by the major histocompatibility complex. In recent years it has become apparent that a minor proportion of malignant lymphomas expresses an NK-cell phenotype defined by its reactivity with the CD56 antibody. Primary purely cutaneous CD56 + lymphomas have rarely been reported. They share a generally aggressive course and are highly associated with Epstein-Barr virus. We describe a patient with a primary cutaneous nasal-type T/NK-cell lymphoma that presented as a haemophagocytic syndrome and showed an aggressive clinical course.

Cutaneous T-cell lymphomas and their management strategies

“…[69] T cell receptor (TCR) α/β cases exhibit an indolent, slowly progressive course compared with γ/δ cases, which have a rapid and aggressive course. [70,71] The prognosis is generally poor, with an overall survival (OS) rate of less than 3 years on aggressive chemotherapy. • Primary cutaneous peripheral T-cell lymphoma, unspecified: This entity includes two subtypes:…”

Extranodal nasal-type natural killer/T-cell lymphoma of the skin: a clinicopathologic study of 16 cases in China

“…To date, some cases of the tumor have been reported in English-language literature, but no related data have come from Mainland China [8,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26]. In the current study, 16 cases of cutaneous ENKTCL-N were studied retrospectively for the clinicopathologic features of the tumor.…”

Nasal-type NK/T-cell Lymphoma Presenting as Hemophagocytic Syndrome in an 11-year-old Mexican Boy

“…It is well established that this condition may present in patients with NK/T lymphoma in both the nasal and nasal-type forms. 11,18 Our patient had evidence of hemophagocytic cells in both the bone marrow and the omental lymph node, indicating the presence of HLH. The visualized hemophagocytic cells had engulfed red blood cells, white blood cells, and other debris.…”

“…6,10,13 There are several reports of patients presenting in their teens, but it is exceedingly rare in the preteen years. 11,[17][18][19] Hemophagocytic syndrome or HLH is an aggressive, life-threatening disease caused by abnormal cytokine dysfunction. There is a proliferation of activated T lymphocytes and histiocytes in multiple organs.…”