Definition of Mature T-Cell Or NK-Cell Neoplasms: A group of neoplasms composed of T-lymphocytes with a mature (peripheral/post-thymic) immunophenotypic profile and/or NK-cells.
2A90 Mature T-cell lymphoma specified types nodal or systemic
2A90.0 T-cell prolymphocytic leukaemia
Definition of T-cell prolymphocytic leukaemia: An aggressive T-cell leukaemia, characterised by the proliferation of small to medium sized prolymphocytes with a mature T-cell phenotype, involving the blood, bone marrow, lymph nodes, liver, spleen, and skin.
2A90.1 T-cell large granular lymphocytic leukaemia
Definition of T-cell large granular lymphocytic leukaemia: A T-cell peripheral neoplasm characterised by a persistent (>6 months) increase in the number of peripheral blood large granular lymphocytes, without a clearly identified cause.
2A90.2 Chronic lymphoproliferative disorders of NK-cells
Definition of Chronic lymphoproliferative disorders of NK-cells: Heterogeneous disorders with a chronic clinical course affecting predominantly adults and characterised by the proliferation of large granular lymphocytes with natural killer cell immunophenotype.
2A90.3 Aggressive NK cell leukaemia
Definition of Aggressive NK cell leukaemia: A rare, highly aggressive, Epstein-Barr virus-associated leukaemia, also known as aggressive NK-cell leukaemia/lymphoma; it may represent the leukemic counterpart of nasal type extranodal NK/T-cell lymphomas. It affects primarily teenagers and young adults. It is characterised by the systemic proliferation of NK cells in the peripheral blood, bone marrow, liver, and spleen.
2A90.4 Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood
Definition of Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood: This neoplasm of childhood is characterised by a clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It can occur shortly after primary acute EBV infection or in the setting of chronic active EBV infection (CAEBV).
2A90.5 Adult T-cell lymphoma or leukaemia human T-cell lymphotropic virus type 1-associated
Definition of Adult T-cell lymphoma or leukaemia human T-cell lymphotropic virus type 1-associated: A peripheral (mature) T-cell neoplasm linked to the human T-cell leukaemia virus type 1 (HTLV-1). Adult T-cell leukaemia/lymphoma is endemic in several regions of the world, in particular Japan, the Caribbean, and parts of Central Africa.
2A90.6 Extranodal NK/T-cell lymphoma nasal type
Definition of Extranodal NK/T-cell lymphoma nasal type: An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of EBV positive NK/T cells. The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder.
2A90.7 Enteropathy associated T-cell lymphoma
Definition of Enteropathy associated T-cell lymphoma: An uncommon mature T-cell lymphoma of intraepithelial lymphocytes. It usually arises from the small intestine, most commonly the jejunum or ileum. Other less frequent primary anatomic sites include the duodenum, stomach, colon, or outside the gastrointestinal tract. Type II of this lymphoma may occur sporadically outside the context of celiac disease.
2A90.8 Hepatosplenic T-cell lymphoma
Definition of Hepatosplenic T-cell lymphoma: An extranodal, mature T-cell non-Hodgkin lymphoma that originates from cytotoxic T-cells, usually of gamma/delta T-cell type. It is characterised by the presence of medium-size neoplastic lymphocytes infiltrating the hepatic sinusoids. A similar infiltrating pattern is also present in the spleen and bone marrow that are usually involved at the time of the diagnosis.
2A90.9 Angioimmunoblastic T-cell lymphoma
Definition of Angioimmunoblastic T-cell lymphoma: A mature T-cell non-Hodgkin lymphoma, characterised by systemic disease and a polymorphous infiltrate involving lymph nodes and extranodal sites. The clinical course is typically aggressive.
2A90.A Anaplastic large cell lymphoma ALK-positive
Definition of Anaplastic large cell lymphoma ALK-positive: A T-cell peripheral lymphoma composed of usually large, pleomorphic, CD30 positive T-lymphocytes with abundant cytoplasm characterised by the presence of a translocation involving the ALK gene and expression of ALK fusion protein. Most patients present with peripheral and/or abdominal lymphadenopathy, and often have advanced disease and extranodal involvement.
2A90.B Anaplastic large cell lymphoma, ALK-negative
Definition of Anaplastic large cell lymphoma, ALK-negative: A T-cell peripheral lymphoma morphologically indistinguishable from anaplastic large cell lymphoma, ALK-positive. It is characterised by the absence of the translocation involving the ALK gene and lacks expression of ALK fusion protein.
2A90.C Peripheral T-cell lymphoma not otherwise specified
Definition of Peripheral T-cell lymphoma not otherwise specified: A heterogeneous category of nodal and extranodal mature T-cell lymphomas, which do not correspond to any of the specifically defined entities of mature T-cell lymphoma in the current classification.
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