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Principles of the WHO classification with special reference to aggressive extranodal lymphomas
Published in Franco Cavalli, Harald Stein, Emanuele Zucca, Extranodal Lymphomas, 2008
Harald Stein, Michael Hummel, Lorenz Trümper, Nancy Lee Harris
Males and females are equally affected, and the median age at presentation is in the third decade of life.143 Aggressive NK-cell leukemia is a rare disease that is found predominantly in Asian countries.144
Choroidal Infiltration as First Clinical Manifestation of T-cell Large Granular Lymphocyte (T-LGL) Leukemia
Published in Ocular Immunology and Inflammation, 2020
Stephanie Sarny, Christine Beham-Schmid, Yosuf El-Shabrawi
Large granular lymphocyte (LGL) leukemia is a lymphoproliferative disease of cytotoxic lymphoid cells. LGLs are T-lymphocytes or natural killer (NK) cells with three LGL subtypes, namely T-LGL leukemia, aggressive NK-cell leukemia, and chronic NK-cell lymphocytosis.1 They are characterized by the accumulation of cytotoxic cells in the blood with infiltration of the bone marrow, the spleen or the liver. T-LGL leukemia is extremely rare with an incidence of 0.2 patients per one million people.2 Men and women are equally affected with a median age of 66 years at diagnosis. A mutation in the STAT3 gene was identified as a recurrent genetic abnormality.3 T-LGL leukemia is associated with lymphocytosis, neutropenia and sometimes anemia and slight thrombocytopenia. Recurrent infections of the upper respiratory due to neutropenia and splenomegaly are the most common clinical manifestations. B-symptomatic is rare and half of the patients are even asymptomatic. Therefore, T-LGL leukemia is primary an indolent disease and some patients do not require systemic therapy over years. However, the disease is frequently associated with autoimmune disorders. Rheumatoid arthritis in up to one-third of all patients is the most common; others are systemic lupus erythematosus, vasculitis or Sjogren syndrome.
Update on the classification of T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms
Published in Expert Review of Hematology, 2019
Akira Satou, N. Nora Bennani, Andrew L. Feldman
EBV-positive T-cell and NK-cell LPDs in the pediatric age group can be categorized into two major groups in the 2017 WHO classification: systemic EBV-positive T-cell lymphoma of childhood and chronic active EBV infection (CAEBV). Systemic EBV-positive T-cell lymphoma of childhood is a clonal proliferation of EBV-infected cytotoxic T cells that shows a fulminant and aggressive clinical course usually associated with hemophagocytic syndrome, multiorgan failure, and sepsis [75,76]. The disease most often occurs in the context of CAEBV, though it can occur shortly after primary acute EBV infection. CAEBV is a systemic EBV-positive polyclonal, oligoclonal, or monoclonal LPD characterized by persistent infectious mononucleosis-like symptoms [77]. Patients with CAEBV show a predisposition to T-/NK-cell lymphoma or aggressive NK-cell leukemia. Kawamoto et al. recently reported that the clinical features of adult-onset CAEBV are distinct from those in pediatric cases, with a decreased incidence of fever but a higher frequency of skin lesions [78]. Of note, adult-onset CAEBV had a poorer prognosis than either pediatric CAEBV or NKTCL.
Dysfunction of immune system in the development of large granular lymphocyte leukemia
Published in Hematology, 2019
Houfang Sun, Sheng Wei, Lili Yang
Large granular lymphocyte (LGL) leukemia is identified as a rare type of lymphoproliferative disorder that results from the clonal expansion of large granular lymphocytes (LGLs) [1–6]. As key innate immune effectors, LGLs occupy roughly 10–15% of the peripheral blood mononuclear cells (PBMCs) and originate from two main lineages, CD3+ cytotoxic T-lymphocytes (CTLs) and CD3- natural killer (NK) cells [7–10]. In 2008, a provisional entity of chronic NK-cell lymphoproliferative disorder (CLPD-NK) was recognized by the WHO in order to distinguish it from the aggressive NK-cell leukemia. Thus there are three kinds of LGL disorders, namely T-cell large granular lymphocyte (T-LGL) leukemia, aggressive NK-cell leukemia and CLPD-NK [1,11,12]. The latest 2016 WHO guidelines followed the previous classification method, but specially underlined the detection of STAT mutations as a new subtype [11,13]. In fact, LGL leukemia can be considered as a result of the clonal expansion of either NK cells or T cells [2]. The abnormal proliferation of LGLs contributes to the development and progression of the immune system disorders in humans [8], including the autoimmune diseases, hematological abnormalities, and dysregulated signal transduction of T cells and NK cells, besides the B-cell dyscrasias. Consequently, LGL leukemia is an excellent model for the study of hematological cancers that manifest immune system dysregulations. Herein we mainly focus on the impaired homeostasis of the immune system in LGL leukemia and briefly introduce the novel therapeutic agents targeting the corresponding abnormalities in LGL leukemia.