비강 T/NK 세포형 림프종 1례

이정복,전인상,임호준,오영하,김지혜,Lee, Jung Bok,Jeon, In-sang,Im, Ho Joon,Oh, Young Ha,Kim, Ji Hye 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.12

저자들은 비강내 종괴로 내원한 14세 환아에서 종괴 생검 및 면역 표현형 검사로 비강 T/NK 세포형 림프종으로 진단하고 치료 중인 1례를 보고하는 바이다. The advance of the immunobiology clarifies the nature of non-Hodgkin's lymphoma(NHL). In addition the proceed in the immunophenotyping renders the classification of NHL. According to the Revised European American Lymphoma(REAL) classification, classified by the etiologic factors, molecular biological characteristics, immunophenotype, cytogenetics and histologic feature, nasal T/NK-cell lymphoma(=angiocentric lymphoma) belongs to the category of peripheral T-cell and natural killer cell lymphoma. Nasal T/NK-cell lymphoma is a distinct clinicopathologic entity characterized by progressive necrotic lesions in the nasal cavity, nasopharynx, and palate. The cellular origin of this tumor has been controversial. Although most nasal T/NK-cell lymphomas are of NK-cell lineage, being CD56+, negative for surface CD3(Leu4), and unassociated with rearrangements of the T-cell receptor genes, other minor variants have been reported. This lymphoma is a rare disease and usually experienced in adult. Recently, we experienced a rare type lymphoma, nasal T/NK-cell lymphoma, in 14 years old boy. His soft mass occupied the right nasal cavity including the nasal septum and turbinate. Pathologically this nasal mass showed the infiltration into the vascular wall, illustrating angiodestructive lesion. The cellular origin was NK-cell lineage, being CD56+ and negative to CD3. Now, we report the case with a brief review of related literatures.

비부비동에 발생한 NK/T 세포 림프종의 예후인자 분석

김동현,윤주헌,강성욱,박정섭,홍성표,강희선,김현준 대한이비인후과학회 2007 대한이비인후과학회지 두경부외과학 Vol.50 No.1

Background and Objectives:Nasal natural killer T (NK/T) cell lymphomas are relatively comon in Asia, but the prognostic factors are not wel known. The purpose of this research was therefore to evaluate those prognostic factors. Subjects and Method:We reviewed and analyzed the medical records of 68 patients diagnosed as nasal NK/T cell lymphomas between 1984 lactate dehydrogenase (LDH) levels, Eastern cooperative oncology group (ECOG) performance status, international prognostic indices (IPI), treatment modality, and Ann Arbor tumor stages were analyzed using the methods of univariate and multivariate statistics. Results:The five-year overall survival rate was 43%. By univariate analysis, we found ECOG performance status, Ann Arbor tumor stages, B symptoms, and IPI to be significant prognostic factors of nasal NK/T cell lymphoma. The multivariate Conclusion:ECOG performance status, Ann Arbor tumor stages, B symptoms, and IPI could all be prognostic factors of the nasal NK/T cell lymphoma. Among these factors, ECOG performance status and B symptoms may be regarded more useful in diagnosis of the disease than others. (Korean J Otolaryngol 2007 ;50 :37-42)

림프절외 림프종의 WHO 분류 : 임상적 및 면역학적인 표현형의 분석 Clinical and Immunophenotypic Analysis

김진만,설지영,강대영 충남대학교 의학연구소 2001 충남의대잡지 Vol.28 No.2

The authors retrospectively reviewed extranodal malignant lymphomas which include various distinctive clinicopathologic entities according to the new WHO lymphoma classification system. The aim of this study was to analyze the clinical characteristics of extranodal lymphomas and the relative frequency of the subtypes with immunophenotype in the Daejeon city and Chungcheong province. The authors reviewed 134 cases of extranodal lymphoma out of 220 cases of total non-Hodgkin's lymphoma which was diagnosed during the last 10 years. Among the cases, gastric lymphomas (26.1%) were the most common anatomic site. Nasal and paranasal (14.4%), skin (13.4%), and Waldeyer's tonsillar ring (11.2%) lymphomas were relatively common in order of frequency. The most frequent subtypes of extranodal lymphoma were marginal zone B-cell lymphoma of MALT (31%), diffuse large B-cell lymphoma (20%), peripheral T-cell lymphoma, unspecified (15.7%), and nasal NK/T cell lymphoma (13.4%), in decreasing order. Compared with a previous studies, the relative frequency of nasal NK/T cell lymphoma was much higher in our study than western countries and similiar to East Asia. The immunophenotype of our extranodal lymphomas was mainly B-cell type (68%) and the remaining cases were T- or NK-cell origin. In conclusion, the occurrence rates for various subtypes of extranodal lymphoma in Daejeon and Chungcheong province were distinct from those in western countries and similiar to those in East Asian countries. The most distincitive entities in our study were marginal zone B-cell lymphoma of MALT and nasal NK/T cell lymphoma. It also appears that the frequency of lymphoma subtypes is changing according to the classification system.

봉소염의 임상양상을 보인 비강의 T / NK - 세포 림프종 1 예

박상진(Sang Jin Park),김수홍(Soo Hong Kim),이일수(Eil Soo Lee) 대한피부과학회 2001 대한피부과학회지 Vol.39 No.8

T/NK-cell lymphoma shows biphenotype of the T-cell and NK-cell. This tumor is characterized by the absence of clonal rearrangement of T-cell receptor gene, frequent association with Epstein-Barr virus(EBV), poor clinical course, and common extranodal invasion. When the tumor did not involve nasal cavity, it is called nasal-type T/NK-cell lymphoma. It commonly appears as deep nodule, infiltrative plaque, and ulcerative plaque. We describe a case of nasal-type T/NK-cell lymphoma, associated with EBV, and showing clonal T-cell receptor y gene rearrangement. The patient was 60-year -old female with a skin lesion on her face, which resembled cellulitis. (Korean J Dermatol 2001;39(8)914-917)

피부를 침범한 비강 원발성 CD56 양성 NK/T-세포 림프종 1예

김태진,김현조,조문균,이성렬,이종석,황규왕 대한피부과학회 2003 大韓皮膚科學會誌 Vol.41 No.1

Recently a distinct subtype of lymphoma was reported, the natural killer(NK)/T-cell lymphoma, which can involve the skin in a primary or secondary event. NK/T-cell lymphoma is characterized by the expression of the NK-cell antigen CD565. CD56+ lymphoma arc further subdivided into nasal and non-nasal NK/T-cell lymphoma. We report a case of primary nasal NK/T-cell lymphoma with secondary cutaneous involvement. (Korean J Dermatol 2003;41(1) : 126∼129)

피부를 침범한 침습성 T/NK-세포 림프종/백혈병

김재왕 ( Kim Jae Wang ),김광중 ( Kim Gwang Jung ),이종주 ( Lee Jong Ju ) 대한피부과학회 2003 대한피부과학회지 Vol.41 No.12

Non-nasal T/NK-cell lymphomas(TNKCL) are further subdivided into primary cutaneous and 4 subtypes of secondary cutaneous lymjphomas such as nasal type, aggressive, blastic(blastoid), and other specific NK-like T-cell lympoma. Aggressive T/NK-cell lymphoma/leukemia(ATNKCL) is a rare leukemic variant of nasal type TNKCL. Although ATNKCL invariably exhibits the identical histomorphological, genotypical and immunophenotyical findings with TNKCL, it is distinguishable from the nasal type of TNKCL in that the former reveals atypical cells having large azurophilic cytoplasmic granules in the peripheral blood and bone marrow. We herein report a rare case of ATNKCL with secondary cutaneous lesions. (Korean J Dermatol 2003;41(12):1638~1643)

괴저고름피부증과 유사한 임상 양상을 보인 비강형 NK/T-세포 림프종 1예

이승훈 ( Seung Hun Lee ),김성민 ( Seong Min Kim ),윤태진 ( Tae Jin Yoon ) 대한피부과학회 2009 대한피부과학회지 Vol.47 No.6

NK/T-cell lymphoma is rare type of lymphoproliferative disorder, but it is clinically aggressive with a poor prognosis. The World Health Organization (WHO) classified mature NK cell neoplasms into extranodal NK/T-cell lymphoma nasal type and aggressive NK cell leukemia. NK/T-cell lymphoma is characterized by a strong association with Epstein-Barr virus (EBV), and when it involved the skin, the lesions clinically appear as deep nodules, infiltrative plaques and ulcerative plaques. We herein report on a case of the nasal type of extranodal NK/T-cell lymphoma that showed the immunophenotype of CD20?, CD56+ and CD45RO+, and it was positive on in situ hybridization for EBV. Our case was characterized by the clinical resemblance with pyoderma gangrenosum and this malady generally displays chronic painful ulcer and necrotic lesions. (Korean J Dermatol 2009;47(6):732∼735)

Case Reports : Mediastinal Single Nodal Relapse of a Nasal Nk/T cell Lymphoma

Kyoung Hoon Rhee,Seok Chan Hong,Jeong Min An,Joo Ryung Huh,,Ryu Jin Sook,Jin Seong Lee,Cheol Won Suh 대한내과학회 2007 The Korean Journal of Internal Medicine Vol.22 No.3

A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma.

비중격 천공에 의해 늦게 진단된 결절외 비성 Natural Killer/T 세포 림프종 1예

이은수,배우용,이창배,박민경 대한이비인후과학회 2020 대한이비인후과학회지 두경부외과학 Vol.63 No.5

Extranodal natural killer (NK)/T-cell lymphoma, nasal type is a rare non-Hodgkin lymphomaoriginating in the nasal cavity or in the paranasal sinuses, and is etiologically closely related withthe Epstein Barr Virus infection. It is more commonly found in East Asia, South America, andMexico than in Europe or North America. Extranodal NK/T-cell lymphoma, nasal type typicallyshows a crust on the nasal mucosal surface, necrosis, and inflammation. Pathologically, itpresents as significant vascular damage and destruction, with apparent tissue necrosis and cytotoxicity. These neoplasms are very aggressive and can show septal perforation, diffuse ulcer lesionon the nasal mucosa, fistula or epistaxis. We experienced a case of the extranodal NK/Tcelllymphoma, nasal type at the nasal septum, which was delayed in diagnosis due to septalperforation, which occurred after septoplasty in a 47-year-old female. Thus, we report this casewith a review of literatures.

Sequential chemotherapy followed by radiotherapy versus concurrent chemoradiotherapy in patients with stage I/II extranodal natural killer/T-cell lymphoma, nasal type

이지은,김철용,이남권,박영제 대한혈액학회 2013 Blood Research Vol.48 No.4

BackgroundThe purpose of this report is to summarize our clinical experience of patients with stage I/II extranodal natural killer (NK)/T-cell lymphoma, nasal type, treated using sequential chemotherapy followed by radiotherapy (SCRT) or concurrent chemoradiotherapy (CCRT).MethodsForty-three patients with stage I/II extranodal NK/T-cell lymphoma, nasal type, who re-ceived SCRT (16 patients) or CCRT (27 patients) were included in the present analysis. ResultsThe median follow-up time was 39 months (range, 4‒171 months) for all patients, 77 months (range, 4‒171 months) for the SCRT group, and 31 months (range, 6‒132 months) for the CCRT group. There were no statistically significant differences between the SCRT and CCRT groups with regard to the 3-year progression-free survival (PFS) (56% vs. 41%, P=0.823) and 3-year overall survival (OS) (75% vs. 59%, P=0.670). Univariate analysis revealed that patients with tumors confined to the nasal cavity and patients achieved com-plete remission had better PFS and OS rates, regardless of the treatment sequence. Multivariate analysis revealed that patients with tumors confined to the nasal cavity and patients aged ≤60 years had better OS rates.ConclusionThe effect of SCRT and CCRT are similar in terms of survival outcomes of patients with stage I/II extranodal NK/T-cell lymphoma, nasal type. Our results show that tumors con-fined to the nasal cavity and an age ≤60 years were associated with a better prognosis.