Differential diagnosis of Wegener's granulomatosis and extranodal NK/T-cell lymphoma, nasal type


Cite item

Full Text

Abstract

Men aged over 40 years more commonly develop NK/T-cell lymphomas (NK/T-CL). The paper describes a case of NK/T-CL in a 20-year-old man. Despite the fact that the disease (nasal septum perforation, hard palate bone destruction, recurrent nasopharyngeal bleeding, considerable weight loss, and high erythrocyte sedimentation rate,) progressed rapidly for 5 months, the patient was found to be diagnosed as having Wegener' granulomatosis (WG). Repeated incisional biopsies showed massive necrotic changes with no clear histological verification of the diagnosis. The absence of lung and kidney lesions typical of WG, the lack of antineutrophil antibodies, and the detection of Epstein-Barr virus DNA in blood could presume NK/T-CL and confirm it by extended biopsy to have materials sufficient for morphological and immunomorphological studies. This observation shows that the disease may occur at a young age and rapidly progress; only early diagnosis can improve prognosis in patients with this type of lymphomas.

Full Text

Дифференциальная диагностика гранулематоза Вегенера с экстранодальной NK/T-клеточной лимфомой назального типа. - Аннотация. NK/Т-клеточные лимфомы (NK/T-КЛ) чаще развиваются у мужчин в возрасте старше 40 лет. Представлен случай развития NK/T-КЛ у пациента в возрасте 20 лет. Несмотря на быстрое прогрессирование заболевания в течение 5 мес (перфорация носовой перегородки, деструкция костей твердого неба, рецидивы кровотечений из носоглотки, значительное снижение массы тела и высокая СОЭ), больной наблюдался с диагнозом гранулематоза Вегенера (ГВ). При неоднократных инцизионных биопсиях выявляли массивные некротические изменения без четкой гистологической верификации диагноза. Отсутствие типичных для ГВ поражений легких и почек, антинейтрофильных антител, а также обнаружение ДНК вируса Эпштейна-Барр в крови позволили предположить NK/T-КЛ и подтвердить ее при расширенной биопсии с получением достаточного для проведения морфологического и иммуноморфологического исследования материала. Настоящее наблюдение показывает, что заболевание может дебютировать в молодом возрасте и быстро прогрессировать; только ранняя диагностика может улучшить прогноз у больных с этим типом лимфом.
×

References

  1. Spits H., Lanier L.L., Phillips J.H. Development of human T and natural killer cells. Blood 1995; 85: 2654-2670.
  2. Oshimi K. Progress in understanding and managing natural killer-cell malignancies. Br J Haematol 2007; 139: 532-544.
  3. Виноградова Ю.Э., Воробьев А.И. Т-клеточные лимфатические опухоли. Руководство по гематологии. Ред. А.И. Воробьева. Т. 2. М: Ньюдиамед 2003: 113-130.
  4. Chan J.K., Jaffe E.S., Ralfkiaer E., Ko Y.H. Aggressive NK-cell leukemia. In: S.H.Swerdlow, E.Campo, N.L.Harris et al. (eds): World Health Organization Classification of Tumors, WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. 4th ed, International Agency for Research on Cancer (IARC), Lyon 2008: 276-277.
  5. Kwong Y.L. Natural killer-cell malignancies: diagnosis and treatment. Leukemia 2005; 19: 2186-2194.
  6. Chan J.K., Yip T.T., Tsang W.Y. et al. Detection of Epstein-Barr viral RNA in malignant lymphoma of the upper aerodigestive tract. Am J Surg Pathol 1994; 18: 938-946.
  7. Cohen J.L., Kimura H., Nakamura S. et al. Epstein-Barr virus-associated lymphoproliferative disease in nonimmunocompromised hosts: a status report and summary of an international meeting. 8-9 September 2008. Ann Oncol 2009; 20: 1472-1482.
  8. Quintanilla-Martinez L., Kremer M., Keller G. et al. p53 mutation in nasal natural killer/T-cell lymphoma from mexico: assotiation with large cell morphology and advanced disease. Am J Pathol 2001; 159 (6): 2095-2105.
  9. Au W.Y., Ma S.Y., Chim C.S. et al. Clinicopathologic features and treatment outcome of mature T-cell and natural killer-cell lymphomas diagnosed according to the World Health Organization classification scheme: a single center experience of 10 years. Ann Oncol 2005; 16 (2): 206-214.
  10. Armitage J.O. A clinical evolution of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. Blood 1997; 89 (11): 3909-3918.
  11. Kwong Y.L. The diagnosis and management of extranodal NK/T-cell lymphoma, nasal-type and aggressive NK-cell leukemia. J Clin Exp Hematopathol 2011; 51 (1): 21-28.
  12. Hasserjian R.P., Harris N.L. NK-cell lymphomas and leucemias; a spectrum of tumors with variable manifistations and immunofenotipe. Am J Clin Pathol 2007; 127 (6): 860-868.
  13. Wong K.F., Chan J.K., Cheung M.M. et al. Bone morrow involvement by nasal NK cell lymphoma at diagnosis is uncommon. Аm J Clin Pathol 2001; 115: 266-270.
  14. Batsakis J.G., Luna M.A. Midfacial necrotizing lesion. Semin Diagn Pathol 1987; 4: 90-116.
  15. Cheung M.M., Chan J.K., Lau V.H. et al. Primary non-Hodgkin lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients. J Clin Oncol 1998; 16: 70-77.
  16. Cohen J.I., Kimura H., Nacamura S. et al. Epstein-Barr virus-associated lymphoproliferative disease in nonimmunocompromised hosts: a status report and summary of an international meeting. Ann Oncol 2008; 20: 1472-1482.
  17. Harris N.L., Jaffe E.S., Stein H. et al. F revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 1994; 84: 1361-1392.
  18. Васильев В.И., Пробатова Н.А., Тупицын Н.Н. и др. Лимфопролиферативные заболевания при болезни Шегрена. Онкогематол 2007; 3: 16-26.
  19. Liang R. Advances in the manangement and monitoring of extranodel NK/T-cell lymphoma,nasal type. Br J Haematol 2009; 147 (1): 13-21.
  20. Suzuki R., Suzumiya J., Yamaguchi M. et al. Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma nasal type. Ann Oncol 2009; 21 (5): 1032-1040.
  21. Lee J., Suh C., Yeon H.P. et al. Extranodal natural killer T-cell lymphoma, nasal-type: a prognostic model from a retrospective multicenter study. J Clin Oncol 2006; 24 (4): 612-618.
  22. Robbins K.T., Fuller L.M., Osborne B. Primary lymphomas of the nasal cavity and paranasal sinuses. Cancer 1985; 56 (4): 814-819.
  23. Gill H., Liang R.H.S., Tse E. Extranodal natural-killer/T-cell lymphoma, nasal type. Adv Hematol 2010: 627401.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download

Copyright (c) 2012 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
 

Address of the Editorial Office:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Correspondence address:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Managing Editor:

  • Tel.: +7 (926) 905-41-26
  • E-mail: e.gorbacheva@ter-arkhiv.ru

 

 © 2018-2021 "Consilium Medicum" Publishing house


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies