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Blastic NK-Cell Lymphoma

Blastic natural killer cell lymphoma (B-NKL) is an extremely rare form of cancer usually occurring in the middle aged or elderly (rarely children). Only a few people are diagnosed with the disease internationally each year. The cause, like in many lymphomas, is unclear but it appears to not be related to the Epstein-Barr virus but may be related to a loss of the RB1 gene or other genetic causes.

The cancer is extremely aggressive and can be resistant to chemotherapy used in other forms of NHL. The disease can appear in the skin or other areas of the body outside of lymph nodes. Cases localized to skin appear to have a better prognosis.

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:

• Abstract: Complete remission in advanced blastic NK-cell lymphoma/leukemia in elderly patients using the hyper-CVAD regimen. Shapiro M et al., Am J Hematol. 2003 Sep; 74(1): 46-51
• Abstract: Successful treatment of relapsed blastic natural killer cell lymphoma with unrelated cord blood transplantation T Yoshimasu et al. Bone Marrow Transplantation (2002) 30, 41-44.
Childhood cutaneous natural killer/T-cell lymphoma successfully treated with only one course of chemotherapy and incomplete tumor resection, Maki Miyazaki et al. NCI, 2001

 

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Resources

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


Survivor Sites:


The Kevin Stump Lymphoma Foundation was formed to help Kevin - a 29 year old blastic NK lymphoma survivor.


References:


Paper on NK Cell Lymphoma by Kazuo Oghimi, International Journal of Hematology, August 2002
Genomic Alterations in Blastic Natural Killer/Extranodal Natural Killer-Like T Cell Lymphoma with Cutaneous Involvement, Xin Mao et al. Journal of Investigative Dermatology 121, 618-627 (2003)


Papers:


Non-B, non-T neoplasms with lymphoblast morphology: further clarification and classification. Karube K et al. Am J Surg Pathol. 2003 Oct; 27(10): 1366-74
DiGuiseppe JA, Louie CD, Williams, JE, et al. Blastic Natural Killer Cell Leukemia/Lymphoma: A Clinicopathologic Study. AM J SURG PATHOL. 1997 Oct;21(10):1223-30


 

Related Articles

MALT lymphomas
Splenic Marginal Zone Lymphoma
Nodal Marginal Zone Lymphoma


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

 

Books

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 17: T-Cell Large Granular Lymphocytic Leukemia, T-Cell Prolymphocytic Leukemia, and Agressive Natural Killer-Cell Leukemia/Lymphoma

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

Chapter 16: Blastic NK-cell Lymphoma

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Did You Know?
Since the early 1970s, incidence rates for non-Hodgkin’s lymphoma have nearly doubled. Incidence rates for Hodgkin’s disease have declined about 60%. Better means of diagnosis has also increased the number as doctors better understand cancer of lymphocytes verses other diseases.
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