Nasal natural killer/T-cell lymphoma
Franco Cavalli, Harald Stein, Emanuele Zucca in Extranodal Lymphomas, 2008
The histology of NK/T-cell lymphoma is characterized by angioinvasion and necrosis, due to the production of Fas cytokines and cytotoxic granules by the malignant NK cells. Indeed, the previous names for the disease were angiocentric lymphoma and lethal midline granuloma. Hence, the clinical features are mainly dominated by local obstructive and destructive symptoms. Since up to two-thirds of cases occur in the upper aerodigestive tract, symptoms included nasal obstruction, ulcer, hemorrhage, pain, and discharge. Patients may present with nasopharyngeal, or oral or palatal ulcers, that were initially biopsied as carcinoma (Figure 14.1).35,36 Advanced local cases may present as hoarseness of voice or difficulty in swallowing or speech. In some cases, intense local inflammation over an apparent small localized disease mass may be sufficient to cause fever of unknown origin or malignant cachexia. Due to the proximity of the nasal area to the orbit, ophthalmological presentations, such as proptosis, diplopia, or even retinal detachment due to effusion, can occur.37 These are more commonly due to intense periorbital inflammation than to direct invasion.
Bone Marrow
Wojciech Gorczyca in Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Hemophagocytic syndrome (hemophagocytic lymphohistiocytosis) is characterized by fever, hepatosplenomegaly, cytopenia, and systemic macrophage activation with hemophagocytosis [4,5]. Blood smear or BM aspirate shows benign histiocytes (macrophages) with phagocytized erythrocytes, platelets, and occasionally granulocytes and lymphocytes (Figure 2.8J). Although it is most commonly associated with inherited or acquired immune deficiencies and viral infections [especially Epstein–Barr virus (EBV)], it can also complicate patients with malignancies. A special form of hemophagocytic syndrome in rheumatic diseases is called macrophage activation syndrome. Among hematopoietic tumors, hemophagocytic syndrome is typical for subcutaneous panniculitis-like T-cell lymphoma, but may also be seen in anaplastic large cell lymphoma (ALCL); extranodal NK/T-cell lymphoma, nasal type; aggressive NK/T-cell lymphoma/leukemia; intravascular large B-cell lymphoma (IVLBCL); plasmablastic lymphoma; and rarely Hodgkin lymphoma (HL).
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
The term angiocentric lymphoma is recommended in the Revised European-American Classification for a histologically similar group of disorders that were previously called lymphomatoid granulomatosis, when the lower respiratory tract was involved, or polymorphic reticulosis and midline malignant reticulosis, for lesions that involved the nose, nasopharynx, palate, and sinuses. These lesions are predominantly localized processes at onset and may be present for years before diagnosis. The histologic findings, particularly in the early stages of disease, are difficult to interpret and may be misdiagnosed as a benign inflammatory processes. A subset of cases of pulmonary lymphomatoid granulomatosis are of B-cell lineage.
Lymphomatoid Granulomatosis in a 14-Year-Old Boy with Trisomy 21 and History of B-Lymphoblastic Leukemia/Lymphoma
Published in Fetal and Pediatric Pathology, 2018
Anna Paulina Matynia, Sherrie L. Perkins, David Li
An infectious etiology should be excluded by cultures and/or special stains in all cases. Inflammatory disorders such as Wegener granulomatosis can enter the differential diagnosis of low grade LYG due to their cellular perivascular infiltrates and necrosis. The presence of true granulomas, multinucleated giant cells, neutrophils and eosinophils support that diagnosis and argue against LYG; however, atypical cells, as seen in our case, are not a part of the infiltrate present in cases of Wegener granulomatosis. Other EBV-associated lymphoproliferative disorders such as lymphoid hyperplasia could also be considered in cases presenting with low grade findings. Notably, low grade EBV associated lesions are not associated with angiodestructive and/or angioinvasive features, present in our case. Other types of EBV associated lymphomas should also be excluded prior to making the diagnosis of LYG. Extranodal NK/T-cell lymphoma, nasal type, may present with angiodestructive growth pattern, and is associated with EBV. The large atypical cells in our case expressed B-cell markers (CD19 and CD20), which help exclude that entity. Classical Hodgkin lymphoma can be considered, especially in the pediatric population, but in our case it was excluded based on morphologic and immunophenotypic finding. In patients with a history of solid organ, bone marrow or stem cell transplant, post-transplant lymphoproliferative disorder (PTLD) would be considered; however, our patient did not have a history of a transplant. Additionally some argue that LYG should not be diagnosed in that clinical setting (4).
Update on the classification of T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms
Published in Expert Review of Hematology, 2019
Akira Satou, N. Nora Bennani, Andrew L. Feldman
Extranodal NK/T-cell lymphoma, nasal type (NKTCL) is the most common of these disorders. NKTCL usually presents in the upper aerodigestive tract and is prevalent in Asia and some areas of Central and South America [24]. A variety of somatic mutations have been identified in NKTCL, most commonly involving the RNA helicase gene DDX3X and members of JAK/STAT signaling pathway (STAT3, STAT5B, JAK3, and PTPRK) [61,72,73]. Recently, Song et al. reported novel activating STAT3 mutations (p.D427H, p.E616G, p.E616K, and p.E696K) and demonstrated for the first time that STAT3 regulates PD-L1 expression in NKTCL [74]. The authors suggested that combining immune checkpoint blockade with STAT3 inhibition might be a promising therapeutic strategy in this disease.
Clinical, endoscopic and pathological characteristics of primary gastrointestinal T-cell and NK/T-cell lymphomas
Published in Scandinavian Journal of Gastroenterology, 2023
Yi Lu, Hailing Liu, Xianhua Zhuo, Tingzhi Liu, Xiaoying Lou, Chujun Li, Min Zhi
The gastrointestinal tract is the most common site of extranodal lymphomas; however, most of them are B-cell lymphomas, especially in the gastric tract [1]. Gastrointestinal T-cell and NK/T-cell lymphomas are relatively rare, and due to their low incidence, heterogeneous behavior, and complex pathological classification, their diagnosis is quite difficult. Gastrointestinal T-cell and NK/T-cell lymphomas mainly include several subtypes: peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), extranodal NK/T-cell lymphoma-nasal type (ENKTCL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), anaplastic large-cell lymphoma, ALK positive (ALCL, ALK+), anaplastic large-cell lymphoma, ALK negative (ALCL, ALK-) and angioimmunoblastic T-cell lymphoma (AITL) [2].
Related Knowledge Centers
- Gastrointestinal Tract
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- Lethal Midline Granuloma
- Epstein–Barr Virus–Associated Lymphoproliferative Diseases
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