<unk>  occurs primarily in Asians and South Americans; it is comparatively uncommon in other areas. Afflicted patients (median age 50–60 years old; males predominate) most often (~80% of cases) present with<unk>  bleeding, upper airway obstruction, perforation of the hard palate, and/or disfiguring, necrotic lesions of the<unk>  cavity, nasopharynx (including Waldeyer's tonsillar ring), paranasal sinuses, palate, and/or eye socket. Less often, patients present with these findings plus signs and symptoms involving extranasal sites such as the skin, upper respiratory tract, gastrointestinal tract, uterus, testes, and/or elsewhere. Rarely, individuals present with evidence of involvement in the later sites without those involving the head/neck area. On further study these individuals may be found to have occult involvement in the head and neck or to develop such involvement. However, ~10 present of patients present with only skin lesions such as a solitary or multiple subcutaneous masses (which may be ulcerated) in the arms or legs while another ~10% present with masses in the lower gastrointestinal tract (which may be accompanied by bleeding or obstruction), salivary glands, testes, muscles, or other organs without evidence of lesions in the head/neck areas. In these cases, there is relatively little involvement of lymph nodes except as a result of direct invasion from non-nodal sites. Thirty-five to forty-five percent of patients present with a history of malaise, fever, night sweats, and/or weight loss. Most (70-75%) patients are diagnosed with early stage I or II disease while the rest have far more serious stage III or IV disease. Rarely, patients with stage III or IV disease have evidence of a life-threatening complication, hemophagocytic lymphohistiocytosis. Also in rare cases, patients evidence a widespread disease that includes malignant<unk>  infiltrations in the liver, spleen, lymph nodes, bone marrow, and/or blood. These case are, or may soon progress to, a related but potentially fatal disease, aggressive<unk> -cell leukemia. About 45% of patients present with elevated levels of serum lactate dehydrogenase; elevation in this serum enzyme is a poor prognostic indicator. Patients with ENKTCL-NT also have elevated levels of plasma EBV DNA. Quantification of these levels at diagnosis correlates with the extent of their tumor load while serially assaying these levels during treatment gives evidence of the tumors response to treatment and residual disease. Rarely, patients show laboratory evidence of hemophagocytic lymphohistiocytosis such as: decreased circulating red blood<unk> s, leukocytes, and/or platelets; increased serum levels of liver-derived enzymes, ferritin, and/or triglycerides; decreased serum levels of fibrinogen; and/or hemophagocytosis, i.e. engulfment of blood<unk> s by tissue histiocytes in the liver, spleen, bone morrow, and/or other tissues. or aggressive<unk> -cell leukemia (e.g. decreased circulating red blood<unk> s, leukocytes, and/or platelets, increased circulating large, granule-containing malignant<unk> <unk> s, and infiltrations of the latter<unk> s in bone marrow and other tissues).
Extranodal NK/T-cell lymphoma, nasal type
Pediatric-type follicular lymphoma
Indolent lymphoma
Pleomorphic T-cell lymphoma
Mature T-cell lymphoma
Primary mediastinal B-cell lymphoma
Nodular lymphocyte predominant Hodgkin lymphoma
T cell/histiocyte-rich large B-cell lymphoma