ANGIOCENTRIC LYMPHOMA WITH GRANULOMATOUS PANNICULITIS IN THE SKIN EXPRESSING NATURAL-KILLER-CELL AND LARGE GRANULAR T-CELL PHENOTYPES

We investigated three patients suffering from angiocentric lymphoma wi th granulomatous panniculitis in the skin. All three patients presente d with multiple purple subcutaneous nodules. Immunohistologically, the lymphoma cells in all three patients expressed CD2 (T-11), CD56 (neur al cell adhesion molecule), and Mik-beta 1 (interleukin-2 beta recepto r). CD3s (CD3, Leu-4)-positive lymphoma cells were found in two patien ts. A pore forming protein (perforin) was detected in the lymphoma cel ls of all three patients. Perforin-possessing lymphoid cells were foca lly scattered in 2 of 15 patients with CD56-negative cutaneous lymphom as who served as controls. By the Southern blot method, one patient sh owed a rearranged T-cell receptor (TcR)beta gene in the biopsied speci men, and the other two patients had germline configurations of TcRs an d immunoglobulin heavy chain genes. One patient had serum anti-human T -cell lymphotropic virus (HTLV)-I antibody, but showed no integration of its proviral DNA. Ultrastructurally, membrane-bound azurophilic gra nules were detected in the atypical lymphoid cells of all three patien ts. Angiocentric lymphoma with panniculitis in three patients showed t he characteristics of natural killer and large granular T-cells. The h istological features might be due to the characteristics of the neopla stic cells with azurophilic granules and perforin.