Cutaneous nonmf tcell and nkcell lymphoproliferative disorders. The two major types of lymphocytes are b cells and t cells, which are derived from pluripotent hematopoetic stem cells in the bone marrow. Posttransplantation lymphoproliferative disorders ptlds of tcell or natural killer nkcell origin are. 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. Clinicopathological states of epsteinbarr virusassociated tnk cell lymphoproliferative disorders severe chronic active ebv infection of children and young adults. Lymphoproliferative disorders lpd12 comprise a heterogeneous group of diseases characterized by uncontrolled production of lymphocytes that cause monoclonal lymphocytosis, lymphadenopathy and bone marrow infiltration.
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. 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. Tcell and nkcell posttransplantation lymphoproliferative disorders article in american journal of clinical pathology 1276. Flow cytometry of t cell lymphoproliferative disorders. The normal cellular counterpart of these processes has been postulated based on functional and immunophenotypic analyses. Epsteinbarr viruspositive tnkcell lymphoproliferative. Refractory coeliac disease type 2 precursor of eatl clonal proliferation of aberrant iels. Lymphoproliferative disorders after hematopoietic stem cell. Primary cutaneous cd4positive smallmediumsized pleomorphic tcell lymphoma. Alternatively, the animals may succumb to ebvlpd despite the presence of antigenspecific t cells if nk cells are required along with. This is a summary report of the consensus meeting held in the 4th asian hematopathology workshop. Posttransplantation lymphoproliferative disorders ptlds of t cell or natural killer nkcell origin are an.
Usefulness of flow cytometry for differential diagnosis of precursor and peripheral tcell and nkcell lymphomas. In the vast majority of patients, there are no clinical symptoms and nklymphocytosis is an. 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. Posttransplant lymphoproliferative disorders of t or nk cell origin t nk ptld are rare entities and their genetic basis is unclear. Overview of ebvassociated tnkcell lymphoproliferative diseases. 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 cell lymphoma di use large b cell lymphoma burkitt lymphoma plasma cell myeloma plasmacytoma other 3. These conditions lie along a continuum of disease and are categorized by the 2008 world health organization classification system as ptld 1. Oct 11, 2012 the current world health organization who separates chronic proliferations of lgl according to their cell lineage into 2 entities. Immature nk cell neoplasms are very rare and include myeloidnk cell precursor acute leukemia and blastic nk cell lymphoma.
T and nkcell lymphoproliferative disorders annals of. Introduction epsteinbarr virus ebv is an oncogenic virus associated with. 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. Subcutaneous, blastic natural killer nk, nktcell, and other cytotoxic lymphomas of the skin. Kanazawa t, hiramatsu y, iwata s, siddiquey m, sato y, suzuki m, et al.
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. 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. Lymphoproliferative disorders after hematopoietic stem cell transplantation. 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. Cutaneous nonmf tcell and nkcell lymphoproliferative. Epsteinbarr virus, korea, lymphoma, lymphoproliferative disease, tnatural killer cell. Tcell and nkcell posttransplantation lymphoproliferative. Phan phd, in atlas of hematopathology second edition, 2008. 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. Research paper genetic landscape of t and nkcell post. Jan 23, 2015 clinicopathological states of epsteinbarr virusassociated tnk cell lymphoproliferative disorders severe chronic active ebv infection of children and young adults. 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.
Tcell and nkcell posttransplantation lymphoproliferative disorders. The umbrella term ebvpositive tnk lymphoproliferative disease in childhoodtype. 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. Review article epstein barr virusassociated tnatural killer. Review article epstein barr virusassociated tnatural. 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. We performed targeted sequencing of 465 cancerrelated genes and highresolution copy number analysis in 17 tptld and 2 nkptld cases. Ebvassociated t and nk cell lymphoproliferative disorders. Appreciation of the genetic and immunologic landscape of these aggressive nhl, including pdl1 b7h1, cd274 expression by. T cell and nk cell lymphoproliferative disorders hematology.
The immunophenotypic attributes of nk cells and nkcell. Tcell lymphoproliferative disorders post thymic tdt ve as compared to prethymic disorders tall tdt positive. 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. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral t cell and nk cell lymphomas. Mature tcell and natural killer nkcell neoplasms represent a heterogeneous spectrum of postthymic tcell and nkcellderived malignancies. Significant geographic variation is noted with increased prevalence of select subtypes i. 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. 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. Chronic lymphoproliferative disorder of natural killer cells.
Peripheral tcell and nk cell lymphoproliferative disorders. T cell and nk cell lymphoproliferative disorders request pdf. Immature nkcell neoplasms are very rare and include myeloidnk cell precursor acute leukemia and blastic nkcell lymphoma. 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. Overview of ebvassociated tnkcell lymphoproliferative. Posttransplant complications including neoplastic disorders such as carcinoma and lymphomas, related primarily to chronic immunosuppression, are well recognised. Hematopoietic stem cell transplantation for adults with.
Lymphoproliferative disorders after hematopoietic stem. Extranodal nk t cell lymphoma, nasal type enktclnt. Tnkcell posttransplant lymphoproliferative disorder of the. As a group, ebvtnklpd are more commonly encountered in asians and native americans from central and south america compared to western populations. 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. Europe pmc is an archive of life sciences journal literature. Jan 01, 2001 immature nk cell neoplasms are very rare and include myeloid nk cell precursor acute leukemia and blastic nk cell lymphoma.
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. Hematopoietic stem cell transplantation for adults with ebv. Ebvpositive t or natural killer nkcell lymphoproliferative disorders ebvtnklpds comprise chronic active ebv disease caebv, severe mosquito bite allergy smba and hydroa vacciniforme hv. Intestinal tcell lymphomas 4 subtypes hepatosplenic t.
The umbrella term ebvpositive tnk lymphoproliferative disease in childhoodtype covers. Lymphoproliferative disorders associated with epsteinbarr virus infection after bone marrow transplantation can be fatal. Expression of cytotoxic proteins in peripheral tcell and natural killercell nk lymphomas. Tnkcell posttransplant lymphoproliferative disorder of. Review indolent t and nkcell lymphoproliferative disorders of the gastrointestinal tract.
Lymphoproliferative disorders of the gastrointestinal. T cell and nk cell lymphoproliferative disorders article in hematology 20011. 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. Intestinal t cell lymphomas 4 subtypes hepatosplenic t. Extranodal nk t cell lymphomanasal type and aggressive nk cell leukemia can also be included in this broad spectrum. 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. Chronic lymphoproliferative disorder of natural killer cells is a rare type of cancer that is generally asymptomatic. 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.
Ebvpositive tnkassociated lymphoproliferative disorders of childhood are a rare but aggressive manifestation of primary ebv infection. Indolent t and nkcell lymphoproliferative disorders of the. Challenges and opportunities for checkpoint blockade in t. Anticcr4 monoclonal antibody mogamulizumab for the treatment of ebvassociated t and nkcell lymphoproliferative diseases. Theres considerable debate among scientists regarding this condition. Tnkcell lymphoproliferative disorders involving blood.
Overall, 377 variants were detected, with an average of 20 variants per case. Ebv t nk lpds include chronic active ebv infection, ebvassociated hemophagocytic lymphohistiocytosis, hydroa vacciniformelike lymphoproliferative disease, and severe mosquito bite allergy. Some patients also exhibit skinrelated symptoms, such as hvlike eruptions or hypersensitivity to mosquito bites. Ebvpositive tcell and nkcell lymphoproliferative disorders of childhood 4 subtypes extranodal nktcell lymphoma, nasal type. Primary gastrointestinal gi t and nk cell lymphomas are usually aggressive neoplasms associated with high morbidity and mortality. T cell neoplasms peripheral t cell lymphoma, nos hepatosplenic t cell lymphoma other 4. Tcell lymphoproliferative disorders are a heterogeneous group of neoplasms with distinct clinicalbiological properties. 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. T cell lymphoproliferative disorders are a heterogeneous group of neoplasms with distinct clinicalbiological properties. Posttransplant lymphoproliferative disorders of t or nkcell origin tnkptld are rare entities and their genetic basis is unclear.
Indolent t and nkcell lymphoproliferative disorders of. Classical hodgkin lymphoma ptld 90% positive abbreviations. These diseases often occur in immunocompromised individuals. Individuals who have some sort of dysfunction with their. Primary gastrointestinal tcell and nkcell lymphoproliferative disorders. Morice, journalamerican journal of clinical pathology, year2007, volume127 6, pages 8816. Ebvpositive t cell and nk cell lymphoproliferative disorders of childhood 4 subtypes extranodal nk t cell lymphoma, nasal type.
Cell lymphoproliferative disorders the t cell lymphoproliferative disorders comprise a variety of entities that result from the clonal expansion of a mature t lymphocyte. Jci are natural killer cells the key to treating epstein. T and b cells that regenerate uncontrollably to produce. 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. Kanavaros p, boulland ml, petit b, arnulf b, gaulard.
We performed targeted sequencing of 465 cancerrelated genes and highresolution copy number analysis in 17 t ptld and 2 nk ptld cases. 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. Indolent t cell lymphoproliferative disorder of the. 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. The current world health organization who separates chronic proliferations of lgl according to their cell lineage into 2 entities. Chronic lymphoproliferative disorder is characterized by a persistent nk cell lymphocytosis 2000. Aug, 2018 chronic lymphoproliferative disorder of natural killer cells is a rare type of cancer that is generally asymptomatic. Ko2 1department of pathology, ewha womans university school of medicine, and 2department of pathology, samsung medical center, sungkyunkwan university school of. Extramedullary disease is more prominent than usually seen in aml with nodal disease in 87. 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. T cell and nk cell posttransplantation lymphoproliferative disorders. 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.
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