Extranodal Natural Killer T-Cell Lymphoma, Nasal Type, With Minimal Osseous Involvement: Report of a Case and Literature Review (original) (raw)
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An unusual case of lymphoma — a case of extranodal NK/T-cell lymphoma, nasal type
The Egyptian Journal of Otolaryngology
Background There is a great geographic variation of extranodal natural killer (NK)/T-cell lymphoma, nasal type (NNKTL) prevalence, with a much higher prevalence in the Asian and South American populations. According to our knowledge and searches, only one other case report/study of NNKTL has been published in South Africa. Case presentation We present a southern-African 31-year-old male residing in a township in the south of Johannesburg, South Africa. He presented with signs and symptoms similar to those of benign upper airway diseases. Further work-up of persisting signs and symptoms yielded a diagnosis of NNKTL. He was treated with the SMILE (steroids — dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide) chemotherapy regimen, and sandwich radiotherapy was planned. He had a partial response to chemotherapy but unfortunately demised due to overwhelming sepsis prior to radiation therapy. Conclusion Making a diagnosis of NNKTL in resource-limited settings is challe...
Diagnostic pathology, 2017
Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare and highly aggressive NK cell neoplasm with a short clinical course and poor prognosis and is often misdiagnosed and confused with NK/T cell lymphoma (NKTL), which has a very different prognosis. Here, we present a case with nasal and bone marrow involvement, provide a literature review and make a differential diagnosis. A 41-year-old male presented nasal congestion pharyngalgia, palatal perforation, high fever and multiorgan dysfunction. Our diagnosis primarily relied on clinical features, the morphology and immunophenotype of the neoplastic cells and imaging studies. Characteristic large granular lymphocytes with azurophilic granules were visible in the bone marrow smears. In addition, the neoplastic cells expressed a typical immunophenotype, and the T cell receptor γ (TCR-γ) gene rearrangement analysis and presence of Epstein-Barr virus (EBV) were negative. The patient's symptoms and signs were temporarily reli...
Nasal-type NK/T-cell lymphoma: a case report
Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2007
Extranodal NK/T-cell lymphoma represents less than 1% of all lymphomas, but is more common in Asia and South America. We present a 67-year-old female with a 10-month history of four reddish-blue firm and painful nodules in the parietal region of the head, ranging in size from 1 to 5 cm. Two nodules were taken for biopsy, which showed atypical lymphoid cells with angiocentric growth pattern. The immunophenotype of the tumor cells was CD45RO +, CD56 +, CD3 + (epsilon chain), CD20-, consistent with the diagnosis of NK/T-cell lymphoma. NK/T-cell lymphomas are rare and the optimal treatment has not been clearly established.
[Extranodal NK/T cell lymphoma of the nasal type]
Revista de la Facultad de Ciencias Medicas, 2002
The vast majority of lymphoma occurring in the testis are diffuse large B-cell Lymphoma. We report on a case of natural killer/T cell lymphoma in a forty four year old male, with bilateral testicular and cutaneous involvement with a highly aggressive course. The tumor cells were positive for both CD56 and Epstein Barr Virus, encoded EBER molecules, and showed focal angiocentric growth thus fullfilling the criteria for NK/T cell lymphoma of nasal type. Dermatopathologists and uropathologists should be aware of this rare entity which may only be diagnosed after extensive inmunohistochemical studies.
An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features
International Journal of Hematology, 2008
Extranodal NK/T cell lymphoma, 'nasal type,' is a rare clinicopathological entity in Europe. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. We report the case of a patient who presented with fever, dyspnea, nasal congestion, headache, distention of right nasal turbinates and exophytic lower leg ulcerating lesions. A CT scan of visceral scull demonstrated a filling mass of right frontal, ethmoidal and maxillary sinuses with erosion of the wall of right maxillary sinus and ventral portion of the diaphragm. A biopsy was performed in the skin lesion and showed an angioinvasive NK/T cell lymphoma CD56 negative with clonal rearrangement of the Tcell-receptor c gene. Up to our knowledge, this is a rare immunophenotype for NK/T-cell, 'nasal type,' lymphomas. However, the lymphoma may be classified as extranodal NK/T cell lymphoma, 'nasal type,' due to typical clinical presentation, radiologic findings and pathological characteristics of polymorphism, angioinvasion, angiodestruction and coagulative necrosis.
The American Journal of Surgical Pathology, 2012
Extranodal NK/T-cell lymphoma (ENKTL), nasal type, may be of NK or T-cell origin; however, the proportion of T-ENKTLs and whether they are of ab or gd type remains uncertain. To elucidate the cell of origin and detailed phenotype of ENKTL and assess any clinicopathologic associations, 67 cases of ENKTL from Thailand were investigated, together with 5 gd enteropathy-associated T-cell lymphomas (EATLs) for comparison. In all, 70% of the ENKTL were T-cell receptor (TCR) b,g and, in cases tested, d negative (presumptive NK origin); 5% were TCR gd + , 3% were TCR ab + , 1% were TCR ab/gd + , and 21% were indeterminate. Out of 17 presumptive NK-ENKTLs tested, 3 had clonal TCR rearrangements. All cases were EBV + and TIA-1 + ; >85% were positive for CD3, CD2, granzyme B, pSTAT3, and Lsk/MATK; and all were CD16 À . Presumptive NK-ENKTLs had significantly more frequent CD56 (83% vs. 33%) and CXCL13 (59% vs. 0%) but less frequent PD-1 (0% vs. 40%) compared with T-ENKTLs. Of the NK-ENKTLs, 38% were Oct-2 + compared with 0% of T-ENKTLs, and 54% were IRF4/MUM1 + compared with 20% of T-ENKTLs. Only ab T-ENKTLs were CD5 + . Intestinal ENKTLs were EBV + and had significantly more frequent CD30, pSTAT3, and IRF4/MUM1 expression but less frequent CD16 compared with gd EATL. Significant adverse prognostic indicators included a primary non-upper aerodigestive tract site, high stage, bone marrow involvement, International Prognostic Index Z2, lack of radiotherapy, Ki67 >40%, and CD25 expression. The upper aerodigestive tract ENKTLs of T-cell origin compared with those of presumptive NK origin showed a trend for better survival. Thus, at least 11% of evaluable ENKTLs are of T-cell origin. Although T-ENKTLs have phenotypic and some possible clinical differences, they share many similarities with ENKTLs that lack TCR expression and are distinct from intestinal gd EATL.
Australasian Radiology, 2004
Two cases of natural killer (NK)/T-cell primary nasal lymphoma with similar clinical presentations are reported, for comparison and contrast, to highlight the clinical issues and challenges posed by this unusual disease, its aggressiveness being matched only by its rarity. Presenting as a lesion in the nasal cavity with histological features of malignant lymphoma, primary nasal lymphoma is an uncommon extranodal lymphoma, which poses problems in both diagnosis and management. In people of oriental descent, the common cell subtype is NK/T-cell. Although it is generally thought that combination treatment with chemotherapy and radiation is the best management for early stage non-Hodgkin's lymphoma (NHL), there is still debate as to whether combined therapy is optimal treatment for this particular subtype of NHL, given that it responds less well to conventional chemotherapy. Herein we report two patients to illustrate these controversies.