Tnkcell caebv has been used in the literature to encompass a very broad spectrum of diseases, including a systemic form which may be polyclonal, fulminant and systemic ebvpositive tcell lpds that are clonal, hv of tcell derivation, and severe mosquito bite allergy usually of nkcell origin. The umbrella term ebvpositive tnk lymphoproliferative disease in childhoodtype. Tnkcell posttransplant lymphoproliferative disorder of the. Refractory coeliac disease type 2 precursor of eatl clonal proliferation of aberrant iels. This is a summary report of the consensus meeting held in the 4th asian hematopathology workshop. Tnkcell lymphoproliferative disorders involving blood. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral tcell and nkcell lymphomas.
Lymphoproliferative disorders lpd12 comprise a heterogeneous group of diseases characterized by uncontrolled production of lymphocytes that cause monoclonal lymphocytosis, lymphadenopathy and bone marrow infiltration. The umbrella term ebvpositive tnk lymphoproliferative disease in childhoodtype covers. Ebvpositive t or natural killer nkcell lymphoproliferative disorders ebvtnklpds comprise chronic active ebv disease caebv, severe mosquito bite allergy smba and hydroa vacciniforme hv. Lymphoproliferative disorders after hematopoietic stem cell. Caebv is defined as an ebvrelated illness characterized by symptoms such as fever, persistent hepatitis, lymphadenopathy, hepatosplenomegaly, pancytopenia, uveitis, and interstitial pneumonia lasting for more than 3 months 3, 12. T and nkcell lymphoproliferative disorders annals of. Posttransplant lymphoproliferative disorders of t or nkcell origin tnkptld are rare entities and their genetic basis is unclear. Posttransplantation lymphoproliferative disorders ptlds of tcell or natural killer nkcell origin are. Green2, bachir alobeid1 and govind bhagat1 1department of pathology and cell biology, division of hematopathology, columbia university medical center, new york, ny usa 2department of medicine, division of digestive. Indolent t and nkcell lymphoproliferative disorders of. Ebvpositive tnkassociated lymphoproliferative disorders of childhood are a rare but aggressive manifestation of primary ebv infection. T cell lymphoproliferative disorders are a heterogeneous group of neoplasms with distinct clinicalbiological properties.
T and nk cell lymphoproliferative disorders of the gastrointestinal gi tract are uncommon, but are important to recognise as there may be morphological and immunophenotypic overlap between lymphoid lesions with vastly different clinical outcomes. Chronic lymphoproliferative disorder of natural killer cells. Expression of cytotoxic proteins in peripheral tcell and natural killercell nk lymphomas. These conditions lie along a continuum of disease and are categorized by the 2008 world health organization classification system as ptld 1. Mature tcell and natural killer nkcell neoplasms represent a heterogeneous spectrum of postthymic tcell and nkcellderived malignancies. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral t cell and nk cell lymphomas. Review article epstein barr virusassociated tnatural killer. Tcell and nkcell posttransplantation lymphoproliferative disorders article in american journal of clinical pathology 1276. Introduction epsteinbarr virus ebv is an oncogenic virus associated with. Jan 23, 2015 clinicopathological states of epsteinbarr virusassociated tnk cell lymphoproliferative disorders severe chronic active ebv infection of children and young adults. Cutaneous nonmf tcell and nkcell lymphoproliferative. Cell lymphoproliferative disorders the t cell lymphoproliferative disorders comprise a variety of entities that result from the clonal expansion of a mature t lymphocyte. The immunophenotypic attributes of nk cells and nkcell.
T nk cell caebv has been used in the literature to encompass a very broad spectrum of diseases, including a systemic form which may be polyclonal, fulminant and systemic ebvpositive t cell lpds that are clonal, hv of t cell derivation, and severe mosquito bite allergy usually of nk cell origin. Primary cutaneous cd4positive smallmediumsized pleomorphic tcell lymphoma. T and nkcell lymphoproliferative disorders of the gastrointestinal gi tract are uncommon, but are important to recognise as there may be morphological and immunophenotypic overlap between lymphoid lesions with vastly different clinical outcomes. We performed targeted sequencing of 465 cancerrelated genes and highresolution copy number analysis in 17 tptld and 2 nkptld cases.
Major risk factors are ex vivo t cell depletion or in vivo t cell depletion with either antithymocyte globulin or monoclonal anti t cell antibodies. Oct 11, 2012 the current world health organization who separates chronic proliferations of lgl according to their cell lineage into 2 entities. Some patients also exhibit skinrelated symptoms, such as hvlike eruptions or hypersensitivity to mosquito bites. Posttransplantation lymphoproliferative disorders ptlds of t cell or natural killer nk cell origin are an uncommon heterogeneous group of lymphoid proliferations that fulfill the criteria for one of the t or nk cell lymphomasleukemias. Overall, 377 variants were detected, with an average of 20 variants per case. Posttransplantation lymphoproliferative disorders ptlds of t cell or natural killer nkcell origin are an.
Other iatrogenic immunodeficiencyassociated lymphoproliferative disorders with a t or nk cell phenotype akira satou,1 toyonori tsuzuki1 and shigeo nakamura2 other iatrogenic immunodeficiencyassociated lymphoproliferative disorders oiialpds with a t or nk cell phenotype are. T and nk cell proliferative diseases associated with acute and chronic epsteinbarr virus ebv infection are uncommon and raise several clinical issues regarding diagnostic criteria and terminology. Challenges and opportunities for checkpoint blockade in t. Tnkcell posttransplant lymphoproliferative disorder of. Review article epstein barr virusassociated tnatural. Tcell and nkcell posttransplantation lymphoproliferative disorders. The tcell lymphoproliferative disorders are a heterogeneous group of nonhodgkins lymphomas nhl for which current therapeutic strategies are inadequate, as most patients afflicted with these nhl will succumb to disease progression within 2 years of diagnosis. Keywords ctcl, gata3, ptcl, tall 1 introduction peripheral tcell lymphoma ptcl, not otherwise specified ptcl, nos and angioimmunoblastic tcell lymphomas aitl, while accounting for the majority of peripheral nkt. The two major types of lymphocytes are b cells and t cells, which are derived from pluripotent hematopoetic stem cells in the bone marrow. Immature nk cell neoplasms are very rare and include myeloidnk cell precursor acute leukemia and blastic nk cell lymphoma. Overview of ebvassociated tnkcell lymphoproliferative diseases.
Epsteinbarr virus, korea, lymphoma, lymphoproliferative disease, tnatural killer cell. Kanavaros p, boulland ml, petit b, arnulf b, gaulard. These diseases often occur in immunocompromised individuals. Review indolent t and nkcell lymphoproliferative disorders of the gastrointestinal tract. Alternatively, the animals may succumb to ebvlpd despite the presence of antigenspecific t cells if nk cells are required along with. Ebvpositive t or natural killer nk cell lymphoproliferative disorders ebv t nk lpds comprise chronic active ebv disease caebv, severe mosquito bite allergy smba and hydroa vacciniforme hv. Nov 20, 2012 t and nk cell proliferative diseases associated with acute and chronic epsteinbarr virus ebv infection are uncommon and raise several clinical issues regarding diagnostic criteria and terminology. Cd30positive lymphoproliferative disorders and a number of rare variants of ctcl including granulamatous slack skin, subcutaneous panniculitic tcell lymphoma, cutaneous lymphoma, nknklike tcell lymphoma, and primary cutaneous cd8positive epidermotropic cytotoxic tcell lymphoma are also considered. Over the past two decades, however, cases of primary gi lymphoproliferative disorders lpds or lymphomas of t or nk cell derivation with indolent behavior have been reported. T cell and nk cell lymphoproliferative disorders hematology.
Chronic lymphoproliferative disorder is characterized by a persistent nk cell lymphocytosis 2000. Individuals who have some sort of dysfunction with their. Indolent t cell lymphoproliferative disorder of the. Extranodal nk t cell lymphomanasal type and aggressive nk cell leukemia can also be included in this broad spectrum. Kanazawa t, hiramatsu y, iwata s, siddiquey m, sato y, suzuki m, et al. Morice, journalamerican journal of clinical pathology, year2007, volume127 6, pages 8816. Europe pmc is an archive of life sciences journal literature. Posttransplantation lymphoproliferative disorders ptlds of t cell or natural killer nkcell origin are an uncommon heterogeneous group of lymphoid proliferations that fulfill the criteria for one of the t or nk cell lymphomasleukemias. Indolent tcell lymphoproliferative disorder of the gi tract may show clinical features similar to those of nkcell enteropathy, and can be readily misdiagnosed as an aggressive peripheral tcell lymphoma. We performed targeted sequencing of 465 cancerrelated genes and highresolution copy number analysis in 17 t ptld and 2 nk ptld cases. Clinicopathological states of epsteinbarr virusassociated tnk cell lymphoproliferative disorders severe chronic active ebv infection of children and young adults. Extramedullary disease is more prominent than usually seen in aml with nodal disease in 87. Lymphoproliferative disorders associated with epsteinbarr virus infection after bone marrow transplantation can be fatal. Tcell lymphoproliferative disorders are a heterogeneous group of neoplasms with distinct clinicalbiological properties.
Posttransplant lymphoproliferative disorders of t or nk cell origin t nk ptld are rare entities and their genetic basis is unclear. Theres considerable debate among scientists regarding this condition. T and b cells that regenerate uncontrollably to produce. Primary gastrointestinal gi t and nk cell lymphomas are usually aggressive neoplasms associated with high morbidity and mortality. T cell and nk cell lymphoproliferative disorders request pdf. Peripheral tcell and nk cell lymphoproliferative disorders. Posttransplant complications including neoplastic disorders such as carcinoma and lymphomas, related primarily to chronic immunosuppression, are well recognised. Lymphoproliferative disorders after hematopoietic stem cell transplantation. Lymphoproliferative disorders after hematopoietic stem. Research paper genetic landscape of t and nkcell post. Tcell lymphoproliferative disorders post thymic tdt ve as compared to prethymic disorders tall tdt positive.
Cutaneous nonmf tcell and nkcell lymphoproliferative disorders. Intestinal tcell lymphomas 4 subtypes hepatosplenic t. Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of lymphocytes into a monoclonal lymphocytosis. Autoimmune disorders or viral infections may be accompanied by a transient increase in circulating nk cells viral stimulus postulated as an activation factor leading to selection of nk cell clones no evidence of direct nk cell infection observed so far may have genetic susceptibility related to haplotypes containing higher numbers of kir. The normal cellular counterpart of these processes has been postulated based on functional and immunophenotypic analyses. Tcell lymphoproliferative disorders that have been reported to be ebv associated include a subset of peripheral tcell lymphomas, angioimmunoblastic tcell lymphoma, extranodal nasal type natural killer tcell lymphoma, and other rare histotypes. Monomorphic ptld monomorphic ptld are monoclonal lymphoid proliferations that meet the criteria for one of the b cell or tnk cell lymphomas recognized in immunocompetent patients.
Problems with ptcl include their rarity, representing usually 1015% of nonhodgkins lymphomas in the western hemisphere, morphologic heterogeneity, and lack of immunophenotypic markers for clonality. As a group, ebvtnklpd are more commonly encountered in asians and native americans from central and south america compared to western populations. Aug, 2018 chronic lymphoproliferative disorder of natural killer cells is a rare type of cancer that is generally asymptomatic. Primary gastrointestinal tcell and nkcell lymphoproliferative disorders. Lymphoproliferative disorders of the gastrointestinal. Hematopoietic stem cell transplantation for adults with ebv. T cell neoplasms peripheral t cell lymphoma, nos hepatosplenic t cell lymphoma other 4. Bcell neoplasms and tnkcell neoplasms which are classi. Anticcr4 monoclonal antibody mogamulizumab for the treatment of ebvassociated t and nkcell lymphoproliferative diseases. Review article epsteinbarr virusassociated tnatural killercell lymphoproliferative disorders sanghui park,1 young h.
Subcutaneous, blastic natural killer nk, nktcell, and other cytotoxic lymphomas of the skin. T cell lymphoproliferative disorders that have been reported to be ebv associated include a subset of peripheral t cell lymphomas, angioimmunoblastic t cell lymphoma, extranodal nasal type natural killer t cell lymphoma, and other rare histotypes. Ebvpositive t cell and nk cell lymphoproliferative disorders of childhood 4 subtypes extranodal nk t cell lymphoma, nasal type. Epsteinbarr virus ebvpositive tcell and natural killer nkcell lymphoproliferative diseases ebvtnklpd are a group of uncommon disorders characterised by ebv infection of t and nkcells. Indolent t cell lymphoproliferative disorder of the gastrointestinal tract or indolent t cell lymphoproliferative disorder of the gi tract itcldgt is a rare and recently recognized disorder in which mature t cell lymphocytes accumulation abnormally in the gastrointestinal tract gi tract. T cell and nk cell lymphoproliferative disorders article in hematology 20011. Extranodal nk t cell lymphoma, nasal type enktclnt. Aggressive nkcell leukemia ebvpositive tcell and nkcell lymphoproliferative diseases of childhood c systemic ebvpositive tcell lymphoma of childhood c chronic active ebv infection of t and nkcell type, systemic form c hydroa vacciniformelike lymphoproliferative disorder c severe mosquito bite allergy extranodal nktcell lymphoma. Intestinal t cell lymphomas 4 subtypes hepatosplenic t. Epsteinbarr viruspositive tnkcell lymphoproliferative. In the vast majority of patients, there are no clinical symptoms and nklymphocytosis is an. Flow cytometry of t cell lymphoproliferative disorders.
Ebvpositive hemophagocytic lymphohistiocytosis ebvhlh and systemic ebvpositive tcell lymphoma of childhood are two direct sequelae of tcell infection by ebv, resulting in a clonal proliferation of cd8. Unlike itcldgt, enktclnt a is an epsteinbarr virusassociated lymphoproliferative disease that commonly involves midline areas of the nasal cavity, oral cavity, and pharynx but may involve the gi tract. Chronic lymphoproliferative disorder of natural killer cells is a rare type of cancer that is generally asymptomatic. Posttransplantation lymphoproliferative disorders ptlds of tcell or natural killer nkcell origin are an uncommon heterogeneous group of lymphoid proliferations that fulfill the criteria for one of the t or nkcell lymphomasleukemias. Appreciation of the genetic and immunologic landscape of these aggressive nhl, including pdl1 b7h1, cd274 expression by. Classical hodgkin lymphoma ptld 90% positive abbreviations. Ebv t nk lpds include chronic active ebv infection, ebvassociated hemophagocytic lymphohistiocytosis, hydroa vacciniformelike lymphoproliferative disease, and severe mosquito bite allergy. Immature nkcell neoplasms are very rare and include myeloidnk cell precursor acute leukemia and blastic nkcell lymphoma. Hematopoietic stem cell transplantation for adults with.
Significant geographic variation is noted with increased prevalence of select subtypes i. Ebvassociated t and nk cell lymphoproliferative disorders. Ebvpositive tnkassociated lymphoproliferative disorders. T cell lymphoma di use large b cell lymphoma burkitt lymphoma plasma cell myeloma plasmacytoma other 3.
Lymphoproliferative disorders with a t or nkcell phenotype akira satou,1 toyonori tsuzuki1 and shigeo nakamura2 other iatrogenic immunodeficiencyassociated lymphoproliferative disorders oiialpds with a t or nkcell phenotype are markedly rare, with only a limited number of cases having been reported thus far. Phan phd, in atlas of hematopathology second edition, 2008. Jan 01, 2001 immature nk cell neoplasms are very rare and include myeloid nk cell precursor acute leukemia and blastic nk cell lymphoma. The current world health organization who separates chronic proliferations of lgl according to their cell lineage into 2 entities.
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