Extranodal natural killer/T-cell lymphoma, nasal type What every physician needs to know: Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) is an aggressive non-Hodgkin lymphoma (NHL) which represents a rare specific extranodal subtype of peripheral T-cell lymphoma (PTCL). ENKL is most commonly seen in Pacific Rim countries and most studies are from the Far East.…
Sézary syndrome What every physician needs to know: Sézary syndrome (SS) is a leukemic form of cutaneous T-cell lymphoma (CTCL), in which patients have significant blood involvement with Sézary cells and diffuse skin erythema (erythroderma). Patients with SS often have shortened survival but life expectancy is still measured in years. Because the disease can progress…
Enteropathy associated T-cell lymphoma What every physician needs to know: Enteropathy associated T-cell lymphoma (EATL) is an aggressive peripheral T-cell lymphoma with a historically poor prognosis. EATL has been described as either arising from a background of celiac disease (type 1) or a monomorphic histology not necessarily related to celiac disease (type 2). Type 1…
Adult T-cell leukemia/lymphoma What every physician needs to know: Adult T-cell leukemia/lymphoma (ATL) is a distinct subtype of mature or peripheral T-cell lymphomas associated with the human T-cell lymphotrophic virus type 1 (HTLV-1). HTLV-1 is a human retrovirus that is endemic in certain parts of the world, including the northern and southern islands of Japan,…
Hepatosplenic T-cell lymphoma What every physician needs to know: Hepatosplenic T-cell lymphoma (HSTCL) accounts for 1.4% of all known peripheral T-cell lymphoma. This is an aggressive lymphoma that primarily affects young adults (median 34 years of age) with a strong male predominance. HSTCL is usually comprised of a clonal expansion of a T-cell subset called…
T-cell prolymphocytic leukemia What every physician needs to know: T-cell prolymphocytic leukemia (T-PLL) is a rare, usually aggressive T-cell malignancy. Like many of the other T-cell lymphomas and leukemias, it is comprised of malignant post-thymic T-cells. The most common presenting features are a markedly elevated lymphocyte count, often with a rapid doubling time, and splenomegaly,…
