T-Cell Lymphomas, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology
Steven M. HorwitzStephen M. AnsellWeiyun Z. AiJeffrey A. BarnesStefan K. BartaJonathan E. BrammerMark W. ClemensAhmet DoğanFrancine M. FossPaola GhioneAaron M. GoodmanJoan GuitartAhmad HalwaniBradley M. HaverkosRichard T. HoppeEric D. JacobsenDeepa JagadeeshAllison JonesAvyakta KallamYoun H. KimKiran A KumarNeha Mehta‐ShahElise A. OlsenSaurabh RajguruSima RozatiJonathan W. SaidAaron C. ShaverLauren SheaMichi M. ShinoharaLubomir SokolCarlos A. Torres‐CabalaRyan A. WilcoxPeggy A. WuJasmine ZainMary A. DwyerHema Sundar
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Abstract:
Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorders arising from mature T cells, accounting for about 10% of non-Hodgkin lymphomas. PTCL-not otherwise specified is the most common subtype, followed by angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, anaplastic lymphoma kinase–positive, anaplastic large cell lymphoma, anaplastic lymphoma kinase–negative, and enteropathy-associated T-cell lymphoma. This discussion section focuses on the diagnosis and treatment of PTCLs as outlined in the NCCN Guidelines for T-Cell Lymphomas.Keywords:
Anaplastic large-cell lymphoma
Large cell
T-Cell Lymphoma
Abstract ALK‐negative anaplastic large cell lymphoma (ALCL) is an aggressive non‐Hodgkin T‐cell lymphoma. Compared to ALK‐positive ALCL, patients with ALK‐negative ALCL typically are older, present with nodal disease, and have lower survival rates. We report a unique presentation of ALK‐negative ALCL in a pleural fluid. Cell block preparation enabled both confirmation of the diagnosis via immunohistochemical staining and gene rearrangement testing for prognostic information.
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Large-cell lymphoma
Pleural disease
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Anaplastic lymphoma kinase negative anaplastic large cell lymphoma (ALK- ALCL) is a definite entity in the WHO 2016 Classification that represents 2-3% of non-Hodgkin lymphoma (NHL) and 12% of T-cell NHL cases. ALK- ALCL lacks ALK protein expression, but expresses CD30 and has morphologic features similar to ALK positive anaplastic large cell lymphoma (ALK+ ALCL). Some studies indicate that ALK- ALCL and ALK+ ALCL possess different molecular and genetic characteristics. Besides, ALK- ALCL is worse than ALK+ ALCL in terms of treatment outcome, prognosis, and long-term survival. This review is aimed at summarizing information about ALK- ALCL, especially with respect to the treatment and prognosis.
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Large-cell lymphoma
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Anaplastic large cell lymphoma (ALCL) is a CD30-positive neoplasm of either T-cell or null-cell lineage accounting for 2% to 8% of all lymphomas.[1][1] Most frequently, patients with ALCL present with advanced disease with extranodal involvement.[2][2] The central nervous system (CNS) (30%),
Anaplastic large-cell lymphoma
T-Cell Lymphoma
Neoplasm
Large cell
Presentation (obstetrics)
Cell lineage
Null cell
Large-cell lymphoma
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The T-cell lymphomas are characterized by marked differences in its global patterns of distribution. The epidemiology and classification of non-Hodgkin lymphoma (NHL) has undergone vast transformation over the past few decades. In an attempt to study the geographical distribution of NHL subtypes, the International NHL Classification Project was conducted in the late 1990s. The 2017 WHO classification broadly divides peripheral T-cell lymphomas (PTCL) into three categories based on their location. They are leukemic (disseminated), nodal, and extranodal. These categories are further subdivided based on morphology, immunohistochemistry, and clinical behavior. Examples of these lymphomas include PTCL not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphomas, anaplastic large-cell lymphoma (ALCL), anaplastic lymphoma kinase positive (ALK+), ALK negative (ALK–) ALCL, intestinal T-cell lymphoma, and breast implant-associated ALCL. This chapter discusses the epidemiological data for each of the individual subtype of PTCL.
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Peripheral T-cell lymphoma
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Anaplastic large cell lymphomas are peripheral T-cell lymphomas that are characterized by a proliferation of large anaplastic blasts expressing CD30. In children, systemic anaplastic large cell lymphomas often present at advanced clinical stage and harbor translocations involving the anaplastic lymphoma kinase (ALK) gene leading to the expression of chimeric anaplastic lymphoma kinase (ALK)-fusion proteins. Primary cutaneous anaplastic large cell lymphoma is regarded as an ALK-negative variant confined to the skin and is part of the spectrum of primary cutaneous CD30-positive T-cell lymphoproliferative disorders. Thirty-three of 487 pediatric patients registered within the Anaplastic Large Cell Lymphoma-99 trial (1999 to 2006) presented with a skin limited CD30-positive lympho-proliferative disorder. In 23 of the 33 patients, material for international histopathological review was available, and the cases were studied for histopathological, immunophenotypical and clinical features as well as for breaks within the ALK gene. Five of 23 cases and one additional case (identified after closure of the trial) expressed ALK-protein. Complete staging excluded any other organ involvement in all children. Expression of ALK proteins was demonstrated by immunohistochemistry in all cases and the presence of breaks of the ALK gene was genetically confirmed in 5 evaluable cases. The histopathological and clinical picture of these skin-restricted ALK-positive lymphomas was indistinguishable from that of cutaneous anaplastic large cell lymphoma. Five children presented with a single skin lesion that was completely resected in 4 and incompletely resected in one. Three of these patients received no further therapy, 2 additional local radiotherapy, and one chemotherapy. All children remain in complete remission with a median follow up of seven years (range 1–8 years). We present 6 pediatric cases of ALK-positive primary cutaneous anaplastic large cell lymphomas. After thorough exclusion of systemic involvement, therapy confined to local measures seems to be sufficient to induce cure.
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Large-cell lymphoma
Lymphoproliferative Disorders
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ALK-negative anaplastic large cell lymphoma (ALCL, ALK－) is a CD30+ T-cell neoplasm composed of large lymphoid cells with abundant cytoplasm and pleomorphic nuclei that lacks expression of ALK protein. We describe a case of ALCL, ALK－with primary involvement of the rectum in a 37 year old man, where the original diagnosis was established based on a colonoscopic biopsy. T-cell lymphomas are rare in the colorectal area and besides ALCL, their differential diagnosis includes enteropathic T-cell lymphoma, peripheral T-cell lymphoma, not otherwise specified, NK/T cell lymphoma, or NK-cell enteropathy. In addition, syncytial variant of classical Hodgkin lymphoma or a pleomorphic CD30-positive diffuse large B-cell lymphoma should also be ruled out. We discuss pitfalls of the differential diagnosis and review the literature of anaplastic large cell lymphoma in the gastrointestinal tract. Correct diagnosis of ALCL in the colon is important to avoid a colorectal surgery for an assumed adenocarcinoma, and to open the possibility for lymphoma-directed chemotherapy.
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Posttransplantation lymphoproliferative disorders (PTLDs) eventually occur in approximately 5% of all organ transplant recipients. Most of cases are B-cell proliferations associated with the Epstein-Barr virus (EBV). T-cell PTLDs are relatively rare, although some estimate that up to 14% of posttransplantation malignant lymphomas are T-cell lymphomas even though only a few of these cases are described in the literature. A literature review found only 77 cases of T-cell PTLD, including 1 case following cardiac transplant, 15 cases associated with EBV, and only 1 case of anaplastic large cell lymphoma (ALCL). This single ALCL case followed a liver transplant, was of the T-cell phenotype, and was EBV negative. In this report, we describe a 14-year-old male who developed an EBV-positive, T-cell PTLD of the ALCL subtype after a period of 14 years following cardiac transplant. Immunohistochemical staining established the T-cell origin of the neoplasm with strong expression of CD45, CD3, CD43, and CD2 and also showed expression of CD30 consistent with the histologic features that suggested ALCL. EBER in situ hybridization detected the presence of the EBV. Polymerase chain reaction analysis for T-cell receptor-gamma gene rearrangements confirmed the T-cell lineage of this lymphoma. To our knowledge, this is the first reported case of an EBV-positive T cell lymphoma of the anaplastic large cell subtype following organ transplant.
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Large-cell lymphoma
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Using pathological and clinical review, to identify all cases diagnosed as peripheral T cell and natural killer (NK) T cell lymphoma over 10 years from one metropolitan Australian hospital.Subtyping was performed using World Health Organization (WHO) 2008 criteria and a comprehensive immunohistochemical panel. Clinical data including follow-up were obtained. There were 47 cases, including 11 peripheral T cell lymphomas, not otherwise specified (NOS), nine extranodal NK T cell lymphomas, nasal type (eight nasal), eight primary cutaneous anaplastic large cell lymphomas, seven angioimmunoblastic T cell lymphomas, three anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphomas, four ALK-negative anaplastic large cell lymphomas, three enteropathic T cell lymphomas and two subcutaneous panniculitis-like T cell lymphomas. Follow-up of 46 of 47 cases (median time 45 months) revealed that 50% (23 of 46) of patients died. Five-year survival rates were: peripheral T cell lymphoma, NOS 39%; angioimmunoblastic T cell, 43%; nasal NK T 67%; ALK-negative anaplastic large cell lymphoma 67% (at 2 years); ALK(+) anaplastic large cell lymphoma 33%; subcutaneous panniculitis-like T cell lymphomas 100%; primary cutaneous anaplastic large cell lymphoma 86%; and enteropathic T cell lymphoma 33% (at 1 year). One patient with Lennert lymphoma suffered four late cutaneous relapses.This first Australian clinicopathological series of peripheral T cell and NK T cell lymphoma shows epidemiological and survival data similar to those for Europe and North America.
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Anaplastic large cell lymphoma (ALCL) is the second most common type of peripheral T cell lymphoma and an aggressive mature T cell lymphoma. About 50 to 70% of systemic ALCLs are anaplastic lymphoma kinase positive (ALK +), the proportion even higher in the pediatric population. The 5-year survival after chemotherapy is around 70 to 80%. But there is a subgroup of ALK+ ALCL patients who are refractory to chemotherapy. Brentuximab vedotin is an approved agent for such patients. The activity of ALK inhibitors in ALK+ non-small cell lung cancer is well known and has been approved for use. The efficacy and safety of ALK inhibitors in ALK + ALCL are largely under-reported. Here we have reported our experience in the use of ALK inhibitors in relapsed refractory ALK+ ALCL.
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Brentuximab vedotin
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Refractory (planetary science)
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