Apr 14, 2017 extranodal natural killer nktcell lymphoma is an aggressive malignancy of putative nkcell origin, with a minority deriving from the tcell lineage. Habermann, md overview primary central nervous system lymphoma, natural killer tcell lymphoma nasal type, and posttransplant lymphoproliferative disorder are uncommon and complex lymphoproliferative disorders. Oct 11, 2012 the current world health organization who separates chronic proliferations of lgl according to their cell lineage into 2 entities. T and nkcell lymphoproliferative disorders annals of.
Extranodal nk t cell lymphomanasal type and aggressive nk cell leukemia can also be included in this broad spectrum. Indolent t and nkcell lymphoproliferative disorders of. Subcutaneous, blastic natural killer nk, nktcell, and other cytotoxic lymphomas of the skin. Ebv has been involved in the occurrence of a wide range of bcell lymphoproliferative disorders lpds, including burkitt lymphoma, classic hodgkins lymphoma, and immunedeficiency associated lpds posttransplant and human immunodeficiency virusassociated lpds. Tcell and nkcell posttransplantation lymphoproliferative. Nivolumab in treating patients with relapsed or refractory.
Nov 26, 2019 this paper summarizes two sessions of the workshop during the xix meeting of the european association for haematopathology eahp held in edinburgh in september 2018 dedicated to lymphomas of the gastrointestinal tract. What are the treatment recommendations for primary. Posttransplant tcell lymphoproliferative disordersan aggressive, late complication of solidorgan transplantation. Lymphoma occur most commonly 80% in the nose and upper aerodigestive tract, less commonly 20% in nonnasal. Frontiers epsteinbarr virusassociated t and nkcell. Indolent t and nkcell lymphoproliferative disorders of the. Background extranasal natural killer nktcell lymphoma and aggressive nkcell leukemia are strongly associated with epsteinbarr virus ebv and most often occur in middleaged individuals. Lymphoproliferative disorders lpds refer to several conditions in which lymphocytes are produced in excessive quantities. Extranodal nkt cell lymphoma and aggressive nk cell. Eber can easily exclude nasal type aggressive extranodal nktcell lymphoma. Systemic t and nkcell lymphoma of childhood overlaps with chronic. Anticcr4 monoclonal antibody mogamulizumab for the treatment.
The tcell lymphoproliferative disorders comprise a variety of entities that result from the clonal expansion of a mature tlymphocyte. Ebvpositive t or natural killer nkcell lymphoproliferative disorders ebvtnklpds comprise chronic active ebv disease. The utility of flow cytometric immunophenotyping in the classification of mature t. 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. Tcell large granular lymphocytic leukemia tcell prolymphocytic leukemia sezary syndrome adult tcell leukemia chronic lymphoproliferative disorders of natural killer nk cells and aggressive nk cell leukemias are the focus of chapter 26 t22. Extranodal natural killer nktcell lymphoma is an aggressive malignancy of putative nkcell origin, with a minority deriving from the tcell lineage. Ebvpositive tcell and nk cell lymphoproliferative diseases of childhood a. Posttransplant lymphoproliferative disorders fact sheet highlights y system. Mar 15, 2019 ebvassociated t and nk cell lymphoproliferative diseases ebv t nk lpds are characterized by the transformation and proliferation of ebvinfected t or nk cells. Abstractsystemic epsteinbarr virus ebvpositive tcell lymphoproliferative disorders lpds of childhood is a highly aggressive ebvpositive t natural killer nkcell lpd, which emerges in the background of chronic active ebv infection caebv or shortly after primary acute ebv infection. Tcell lymphoproliferative disorders are among the most challenging.
Listing a study does not mean it has been evaluated by the u. Biological functions of t and nk cells and ebv infection. Ebvpositive tnk lpd encompasses disease entities with a. Epsteinbarr virusassociated t and nkcell lymphoproliferative. Intestinal tcell lymphomas 4 subtypes hepatosplenic t.
Gene mutations in lymphoproliferative disorders of t and nkt. As for many other types of tcell and nkcell neoplasms. T and nkcell lymphomas and systemic lymphoproliferative. Ebvpositive t cell and nk cell lymphoproliferative disorders of childhood 4 subtypes extranodal nk t cell lymphoma, nasal type. Ebvpositive tcell and nkcell lymphoproliferative disorders of childhood 4 subtypes extranodal nktcell lymphoma, nasal type. Cldnk, which has also been known as the nktype lymphoproliferative disorder of granular lymphocytes, is generally considered indolent. Epsteinbarr viruspositive tnkcell lymphoproliferative. Primary gastrointestinal tcell and nkcell lymphoproliferative disorders enteropathyassociated t cell lymphoma eatl and precursor lesions type 2 refractory coeliac disease, rcd monomorphic epitheliotropic intestinal t cell lymphoma indolent t cell lymphoproliferative disorders of the gastrointestinal tract nk cell enteropathy. Ko2 1department of pathology, ewha womans university school of medicine, and 2department of pathology, samsung medical center.
Hematopoietic stem cell transplantation for adults with ebvpositive t or nk cell lymphoproliferative disorders. Flow cytometry of t cell lymphoproliferative disorders. Type, and posttransplant lymphoproliferative disorder tracy t. Epsteinbarr virusassociated lymphoproliferative disorders.
Epsteinbarr virusassociated lymphoproliferative diseases. Mature t and nk cell lymphoproliferations mature tcell leukemias chronic lymphoproliferative disorders of nk cells published date. Ebvpositive systemic tcell lymphoproliferative disease stlpd of childhood, and chronic active ebv caebv infection may develop after primary ebv infection. Chronic lymphoproliferative disorder of nk cells 1856. 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. Anticcr4 monoclonal antibody mogamulizumab for the. Primary gastrointestinal tcell and nkcell lymphoproliferative disorders. Jun 28, 2019 other iatrogenic immunodeficiencyassociated lymphoproliferative disorders oiialpds with a t or nk cell phenotype are markedly rare, with only a limited number of cases having been reported thus far. The t cell lymphoproliferative disorders comprise a variety of entities that result from the clonal expansion of a mature t lymphocyte. Extranodal nk t cell lymphoma, nasal and nasal type is more prevalent in asia and south and central america.
A high definition picture of somatic mutations in chronic. And patient has primary cutaneous anaplastic large cell lymphoma alcl with. The pathogenesis of tcell and nkcell caebv is uncertain. Indolent t cell lymphoproliferative disorder of the. Aug, 2018 chronic lymphoproliferative disorder of natural killer cells is a rare type of cancer that is generally asymptomatic. What are the treatment recommendations for primary cutaneous. None of the 59 enrolled patients was reported to have an episode of hypersensitivity to mosquito bites.
T cell and nk cell lymphoproliferative disorders hematology. Overview of ebvassociated tnkcell lymphoproliferative diseases. Tcell posttransplantation lymphoproliferative disorders after cardiac transplantation. Massone c, chott a, metze d, kerl k, citarella l, vale e, et al. In addition, pathologic findings for these 2 diagnoses are typically notable for necrosis, apoptosis, angioinvasion, and angiodestruction. Ebvassociated t and nkcell lymphoproliferative diseases ebvtnk lpds are characterized by the transformation and proliferation of ebvinfected t or nk cells.
The first session focused on the clinical and pathological features of primary gastrointestinal t cell and nkcell lymphoproliferative disorders. Refractory coeliac disease type 2 precursor of eatl clonal proliferation of aberrant iels. Improved outcomes for extranodal natural killer tcell. Gene mutations in lymphoproliferative disorders of t and. Batchelor, md, mph, lim soon thye, md, and thomas m. Expression of cytotoxic proteins in peripheral tcell and natural killer cell nk lymphomas. The cutaneous nasal type nktcell lymphomas occur more often in females, have a higher frequency of a tcell rather than a nkcell phenotype and less association with ebv. Tcell and nkcell posttransplantation lymphoproliferative disorders. Publications home of jama and the specialty journals of. The tumour nk cells usually express brightly the cd56 adhesion molecule and cd94 lectin type killer receptor, and have an activationrelated cytoplasmic cd3. Epsteinbarr virusassociated t and nkcell lymphoproliferative diseases. They typically occur in people who have a compromised immune system. An approach to diagnosis of tacell lymphoproliferative disorders by. Indolent tcell proliferative disorder of the git is a rare and.
In addition, the progressionfree survival rate will be reported. Extranodal nktcell lymphoma, nasal type and other types of peripheral tcell lymphomas may also occasionally occur in the gi tract 7,8. Emphasis will be placed on extranodal t cell and natural killer nk cell lymphomas such as hepatosplenic lymphoma, subcutaneous panniculitislike lymphoma and nasalnasal type tnkcell lymphoma. Hematopoietic stem cell transplantation for adults with. Posttransplantation lymphoproliferative disorder ptlpd is characterized by lymphoid proliferation after organ or bone marrow transplantation. Some types of primary immunodeficiencies are well known because their main feature is the development of ebvassociated disease. Epsteinbarr virus ebv infects not only b cells but also t cells and natural killer nk cells, and t and nkcell lymphoproliferative diseases tnklpd that are refractory to conventional chemotherapies may develop. Mature tcell and natural killer nkcell neoplasms represent a heterogeneous spectrum of postthymic tcell and nkcellderived malignancies. Epsteinbarr viruspositive tnkcell lymphoproliferative disorders. The t cell variations are usually caused by the prolonged use of t cell suppressant drugs, such as sirolimus, tacrolimus, or ciclosporin.
Ebv is also associated with diseases of t and nkcell origin, such as extranodal nk tcell lymphomanasal type enktl, aggressive nkcell leukemia ankl, and ebvt nklpds as already mentioned. Extranodal nktcell lymphoma, nasal type and other types of peripheral tcell lymphomas may. The definition of the disease allows for a true nk or true t. However, the specific t or nk cell receptors that contribute to the disease state are not known. Lgl is a chronic clonal lymphoproliferative disorder that can be phenotypically subdivided into tcell lgl and natural killer lgl. Although understanding of the heterogeneous landscape of natural killer nkcell malignancies remains limited, extranodal nk tcell lymphoma enktl stands out globally as a distinct disease entity. They are sometimes equated with immunoproliferative disorders, but technically lymphoproliferative disorders are a subset of immunoproliferative disorders, along with hypergammaglobulinemia and. The distribution of progressionfree survival will be estimated using the method of kaplanmeier. Pcnktl affected face, neck, or limbs but not trunk, with a better outcome. Mature t and nk cell lymphoproliferations mature t cell leukemias chronic lymphoproliferative disorders of nk cells published date. The distinction between precursor lesions rcd type 2 and enteropathyassociated t cell lymphoma were stressed, including the discussion of new diagnostic markers for the identification of aberrant. Ebvassociated tcell and natural killer cell lpds ebv is a ubiquitous herpes virus with tropism for b cells, but the infection of t cells and natural killer nk cells may lead to several ebvrelated lpds. The 2016 revised world health organization classification recognizes the following ebvpositive lymphoproliferative disorders lpd.
The first session focused on the clinical and pathological features of primary gastrointestinal t cell and nk cell lymphoproliferative disorders. The immunophenotypic attributes of nk cells and nkcell. Immunodeficiency plays a key role in the pathogenesis of some lymphoproliferative disorders lpds. Posttransplantation lymphoproliferative disorders ptlds of tcell or natural killer nk cell origin are an uncommon heterogeneous group of lymphoid proliferations that fulfill the criteria for one of the t or nkcell lymphomas leukemias. Chronic active ebv infection of t and nkcell type i. Ebvpositive t cell and nk cell lymphoproliferative diseases of childhood c systemic ebvpositive t cell lymphoma of childhood c chronic active ebv infection of t and nk cell type, systemic form c hydroa vacciniformelike lymphoproliferative disorder c severe mosquito bite allergy extranodal nk t cell lymphoma, nasal type intestinal t cell. Chronic lymphoproliferative disorder of natural killer cells or chronic nkcell lymphocytosis is a rare type of potentiallycancerous condition that is generally asymptomatic. The diagnosis and management of nktcell lymphomas journal. Chronic lymphoproliferative disorders of nk cells clpdnk, provisional entity are characterized by a persistent 6 months increase in peripheral blood nk cells usually. Kinney gives a general overview of the diagnosis of ptcl and nk cell neoplasms. 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. Pathologically, the malignancy occurs in two forms, extranodal nktcell lymphoma, nasal type.
Spectrum of t and nk lymphoproliferative disorders in. Immunodeficiency is caused by aging, primary immune disorders, hiv infection, and immunosuppressive drugs. In most reported cases, these cause b cell lymphoproliferative disorders. Flow cytometry applications in the diagnosis of tnk. Mar 09, 2017 nivolumab in treating patients with relapsed or refractory peripheral t cell lymphoma the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Primary gastrointestinal tcell and nk cell lymphoproliferative disorders enteropathyassociated t cell lymphoma eatl and precursor lesions type 2 refractory coeliac disease, rcd monomorphic epitheliotropic intestinal t cell lymphoma indolent t cell lymphoproliferative disorders of the gastrointestinal tract nk cell enteropathy. B cells, t cells, nk cells, and histiocyticdendritic cells, are infected with the epsteinbarr virus ebv. Lymphoproliferative disorders lpds are disorders in which there is uncontrolled production of excess lymphocytes due to several reasons. Systemic ebvpositive tcell lymphoma of childhood previously lpd, aggressive nkcell leukemia, extranodal nktcell lymphoma, nasal type.
Ebvpositive lymphoproliferations of b t and nkcell derivation. Ebvpositive tnkcell lymphoproliferative disease of. Intestinal t cell lymphomas 4 subtypes hepatosplenic t. Nk t proliferations are very rare and only a few cases have been described in children, most of them nktcell lymphoma or leukemia, but not nasaltype nk tcell lymphoma. Nkt proliferations are very rare and only a few cases have been described in children, most of them nktcell lymphoma or leukemia, but not nasaltype nktcell lymphoma. Following the screening of 57 clpd nk patients, only five. The world health organization who classification of tumours of haematopoietic and lymphoid tissues stratifies neoplasms according to lineage and state of differentiation of the neoplastic cells. The who classification considers lymphoproliferative disorders under three broad groupings of b cell neoplasms, tcell and nkcell neoplasms, and hodgkin lymphoma. Ebvpositive tnk cells have been identified in the tonsils and peripheral blood from patients with infectious mononucleosis 17, and the virus has been shown to infect nk cells in vitro18. Some patients also exhibit skinrelated symptoms, such as hvlike eruptions or hypersensitivity to mosquito bites. Although the cell of origin is often a nk cell, particularly in patients in the far east. Theres considerable debate among scientists regarding this condition. Jan 04, 2019 ebv t nk lpds include chronic active ebv infection, ebvassociated hemophagocytic lymphohistiocytosis, hydroa vacciniformelike lymphoproliferative disease, and severe mosquito bite allergy. The most common types reported are peripheral tcell lymphoma, unspecified, and hepatosplenic tcell lymphoma.
T cell and nk cell lymphoproliferative disorders request pdf. Tcell lymphoproliferative disorders post thymic tdt ve as compared to prethymic disorders tall tdt positive. It consists of the tissues and organs that a lymphoproliferative disorder is a disease in which cells of the lymphatic system grow uncontrollably. Significant geographic variation is noted with increased prevalence of select subtypes i. Primary cutaneous nktcell lymphoma of nasal type pcnktl was reported. An unusual case report of indolent tcell lymphoproliferative.
Review article epsteinbarr virusassociated t natural killer cell lymphoproliferative disorders sanghui park,1 young h. Extranodal natural killertcell lymphoma, nasal type. Review article epstein barr virusassociated tnatural. Hematopoietic stem cell transplantation for adults with ebv. These cells play critical roles in the immune system and in the regulation of immune responses.
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