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Extranodal NK or T Cell Lymphoma - Nasal

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The extranodal nasal NK or T Cell lymphomas are rare forms  of lymphoma.   Nasal-type T/NK-cell lymphomas are common in  Asia and in parts of Latin America but rare in the United States and in Europe. This may reflect an ethnic predisposition for the disease. Children may be effected as well as adults. Most   studies have shown a male-to-female ratio of 2:1 to 3:1  

NK or T cell nasal lymphomas represent about 75% of all nasal lymphomas, the rest being B-cell lymphoma.  Tumors are most common in the nasal cavity but other sites may include the skin, gastrointestinal tract, testis, kidney, upper respiratory tract and rarely the eye/orbit.  Commonly patients present with a nasal  mass with bleeding and local bony destruction. Rarely they may present with skin ulcer or GI perforation if these sites are primarily involved.  About 10-20% of patients presenting with nasal NK/T-cell lymphoma may also have skin involvement at same  time.
 

The course of the disease is usually aggressive, with a large number of Asian studies reporting a median survival of 6 to 25 months. Several American and European studies have shown similar results.

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The cause, like in many lymphomas, is unclear but it appears to  be related to the Epstein-Barr virus.   

Treatment:

The prognosis of extranodal NK/T-cell lymphoma is variable. Some patients respond well to chemotherapy such as CHOP combined with local radiation, achieving complete remission and others perish with disseminated disease despite aggressive therapy.  Some have suggested use of daunorubicin chemotherapy or integration of L-asparaginase into therapy.  Transplants also appear to have some promise in treatment.

Since this cancer is so rare, your medical team will most likely search the current medical literature such as PubMed for the  latest successful treatment strategies.  Below are two example abstracts on treatments used from the literature:

Further Reading / In-Depth

Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004.

  • Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section
    • Chapter 26: Mature Nodal and Extranodal T-Cell and Non-Hodgkin's Cell Lymphomas (including Nasal Natural Killer/T-Cell Lymphoma)

Excellent but Technical: An Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed, 2005

  • Chapter 6: Other Cutaneous Cytotoxic Lymphomas
    • Extranodal NK/T-Cell Lymphoma, Nasal Type pp. 73-75

Web Resources:

  • Paper on NK Cell Lymphoma by Kazuo Oghimi, International Journal of Hematology, August 2002

References:

Papers:

  • Jaffe ES, Harris NL et al. WHO Classification of tumors, Tumors of Hematopoietic and lymphoid tissues, IARC Press, 2001, Chapter 7, 204-207
  • Barnes L, Swerdlow S. Surgical Pathology of the Head and Neck, 2001, Marcel Dekker, Inc. New York, Vol 2, Chapter 18, 1307-1312,
  • Greer GP, Kinney MC et al, T cell and NK cell lymphoproliferative disorders, Hematology, American Society of Hematology, 2001
  • Yong W, Zheng W et al, Clinical characteristics and treatment of midline nasal and nasal type NK/T-cell lymphoma, Zhonghua Yi Xue Za Zhi 2001 July; 81(13): 773-5
  • Cheung MM, Chan JK et al, Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients. J. Clin Oncol 16:70-77.

Non-Hodgkin's Lymphoma Information Pages:

    Non-Hodgkin's Lymphomas
    Non-Hodgkin's Lymphoma: Diagnosis
    Non-Hodgkin's Lymphoma: Aggressive Lymphomas
    Non-Hodgkin's Lymphoma: Indolent Lymphomas
    Non-Hodgkin's Lymphoma: Treatment
    Non-Hodgkin's Lymphoma: Resources
    Bone Marrow and Stem Cell Transplants

Childhood Lymphoma Information Pages:

    Childhood Lymphoma
    Childhood Non-Hodgkin's Lymphoma
    Childhood Non-Hodgkin's Lymphoma: Diagnosis
    Childhood Non-Hodgkin's Lymphoma: Types and Staging
    Childhood Non-Hodgkin's Lymphoma: Treatment
    Childhood Lymphoma: Resources

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This page is a work in progress - if you have more complete information, references, or other information please contact the author.  The author is not in the medical field and does not warrant the correctness of the material on this page or the sites linked - please take online information and consult with your own medical team to make informed decisions.

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Updated October 30, 2005