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Extranodal NK-T-cell lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Synonyms and keywords: Angiocentric T cell lymphoma; Extranodal NK-T-cell lymphoma, nasal type; Extranodal NK-T-cell lymphoma, extra nasal type


Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

Overview

Extranodal NK cell lymphoma probably was first reported by McBride as a disease which rapidly destructs nose and face with progressing necrotic granuloma. The natural history of extranodal NK cell lymphoma was generally aggressive and lethal, this disease was initially termed as “rhinitis gangrenosa progressiva”. Since the lesions usually was in midline and was aggressive and lethal, the term “lethal midline granuloma (LMG)” was used. First known case of natural-killer-cell lymphoma was diagnosed in a 19-years old boy. The diagnosis of natural-killer-cell lymphoma was confirmed by pathology as Wegener’s Granulomatosis was ruled out.In contrast with B-cell lymphoma, the classification of such a rare neoplasm has been controversial, since the cytologic features have not been very useful. Further, by many entities, T-cell and natural killer cell (NK) neoplasms do not share any similar immuno-phenotype. Because of such matters, clinical features became handier for classification and somehow even more important than the precise cell of origin. since the majority of cytotoxic T-cell and NK cell lymphomas are located out of lymph nodes, the gene expressing cytotoxic molecules may predispose to apoptosis by tumor cells and by standard cells.Three major categories of extranodal T/NK cell tumors include: Extranodal NK/T-cell lymphoma, nasal type lymphoma, Extranodal NK/T-cell lymphoma, enteropathy type lymphoma, Extranodal NK/T-cell lymphoma, subcutaneous panniculitis-like, Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes:, NK cell-derived neoplasms, namely, aggressive NK cell leukemia, Extranodal NK/T-cell lymphoma, nasal type, Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into: Extranodal NK-T-cell lymphoma, nasal type, Extranodal NK-T-cell lymphoma, extra nasal type.NK cells are CD3 and myloperoxidase negative on their surface. NK cells have germline configuration of T-cell receptor and immunoglobulin genes. NK cells originate from a bipotent NK/T-progenitor cell so they have a lot in common with T cells. NK cells express T-associate markers such as CD2, CD3e, CD7, CD8, CD16, CD56, CD57. Most tumors involve NK cells but also involve T cells as neoplastic cells, that is the main reason of classification as NK/T-cell lymphoma rather than NK-cell lymphoma. the immunophenotype of NK lymphoma cells is classically positive for CD2, CD56, and cytoplasmic CD3 epsilon. they are negative for surface CD3. Unlike normal NK cells, the tumor cells are usually negative for CD7 and CD16. they express cytotoxic granule associated proteins granzyme B, T-cell restricted intracellular antigen (TIA-1), and perforin. NK/T cell lymphoma expressing CD56 is rare but most commonly occurs int he head and neck region, skin, and soft tissue. genes involved in the pathogenesis of extranodal NK-T-cell lymphoma include.:HLA DQA1*0501, HLA DQB1*0201. these genes also are relevant with celiac disease.On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma.Coagulative necrosis and apoptotic bodies are frequently encountered., Extranodal NK/T-cell lymphoma, nasal type occurs in nasal cavity and upper aerodigestive tract., Extranodal NK/T cell lymphoma, nasal type affects the nose and facial mid line exhibiting aggressive destruction., on microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma. the tumor cells are small to medium in size with occasional large and anaplastic forms. the lymphoma cells may be admixed with a polymorphic infiltrate of nonneoplastic inflammatory cells including small lymphocytes, plasma cells, histiocytes, and eosinophils.Extranodal NK/T-cell lymphoma is caused by transition mutation of p53. P53 overexpresion has assosiation with poor prognosis.Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as NK cell lukemia, lymphomatoid granulomatosis, EBV-positive diffuse large B cell lymphoma, NOS, anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, peripheral T cell lymphoma, hepatosplenic T cell lymphoma. Extranodal NK/T-cell lymphoma, nasal type must be diffrentiated from Wegner’s granulomatosis, Polymorphic reticulosis, and Midline malignant lymphoma.NK cell lymphoma shows a poor prognosis because of rapid local progression and distant metastasis.The median age of onset is approximately 50 years and it is common in elderly. extranodal NK/T-cell lymphoma is a rare disease in children and often it is associated with mosquito-bite hypersensitivity or other EBV-associated disease.Natural Killer (NK) cell lymphoma is a rare disease. extranodal NK/T cell lymphoma, nasal type (NKTCL) and aggressive NK-cell leukemia (ANKCL) have a higher incidence in Asia, Central, and South America.NK T cell lymphoma, nasal type (NNKTL) consist 3000-10000 out of 100000 cases of non-Hodgkin lymphoma in Asia and South America and less than 1000 in 10000 patient in western countries.There is a strong association between with extranodal NK/T-cell lymphoma and EBV virus.There is a strong assosiation between Extranodal NK/T-cell lymphoma andFas(Po-1/CD95) mutations.According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.Extranodal NK/T cell lymphoma survival rate is not well predicted by Ann Arbor staging system. A new system was introduced based on B symptoms, Ann Arbor system, LDH level, and regional lymphadenopathy combined. Another parallel mechanism for extranodal NK/T cell lymphoma is based on tumor biologic and micro-environmental factors. High Ki-67 nuclear antigen is a marker for actively proliferation tumor cell which could be a related factor with mass bulk.

combination of these two findings on immunophenotyping of frozen section specimen cells and RT-PCR are confirmatory for Extranodal NK/T cell lymphoma:Detection of CD56 and EBER1 on tumor cells and EBV DNA (RT-PCR).Pathological examination of frozen section specimen may misled the NK/T cell diagnosis.Circulating cell free EBV DNA levels by RT-PCR is a diagnostic factor for EBV associated malignancies, Like extranodal NK/T cell lymphoma. Measuring both BamHI W DNA and LMP1 DNA is more useful as a prognosis predictor, these DNAs will decrease with treatment and increase with relapse. Recently, determination of negative immune checkpoints are considered as a drug target,in the case of extranodal NK/T cell lymphoma, PD-L1 will be expressed in the tumor, and patients with higher concentration of soluble PD-L1 showed worse prognosis.

The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Extranodal NK/T cell lymphoma, nasal type, which is the most common sub type, is usually found as an ulcerative and necrotic granuloma in the nasal cavity, palate, and nasopharynx. Tumor can spread to surrounding tissue such as facial skin, paranasal sinus, and orbits, and cause exensive destruction of midline lesions. The most common symptoms at the time of diagnosis are nasal obstruction and bloody rhinorea.

Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.

Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.

X-rays graphy of patients with extranodal NK/T cell lymphoma is not very usefull in order of diagnosis. Radiography show non-specific tumor changes.

CT scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma. Computerized tomography (CT) may be performed to detect metastasis and assess the local extent of extranodal NK-T-cell lymphoma.

MRI may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.Magnetic resonance imaging (MRI) may be performed to better define local soft tissue and bony involvement of extranodal NK-T-cell lymphoma.

The predominant therapy for extranodal NK-T-cell lymphoma is radiation therapy. Adjudicative chemotherapy and stem cell transplant may be required.

Surgical intervention is not recommended for the management of extranodal NK/T cell lymphoma.

There are no established measures for the primary prevention of extranodal NK/T cell lymphoma.

Effective measures for the secondary prevention of extranodal NK-T-cell lymphoma include: LDH , MLH1, PDGFRA, VEGF, PD-L1, PD-1, Cyclin D1, p53, Ki-67.

High dose chemotherapy and autologous haematopoietic stem cell transplantation is used for varying types of lymphoma, however there is a lac of such experience in treating extranodal NK-T cell lymphoma. Further clinical trials are needed to prove that this therapy is handy and can be used as a method of therapy. Autologous transplantation appears to provide a great survival benefit only for those who attended a complete remission at the time of transplantation.

Historical Perspective

Extranodal NK cell lymphoma probably was first reported by McBride as a disease which rapidly destructs nose and face with progressing necrotic granuloma. The natural history of extranodal NK cell lymphoma was generally aggressive and lethal, this disease was initially termed as “rhinitis gangrenosa progressiva”. Since the lesions usually was in midline and was aggressive and lethal, the term “lethal midline granuloma (LMG)” was used. First known case of natural-killer-cell lymphoma was diagnosed in a 19-years old boy. The diagnosis of natural-killer-cell lymphoma was confirmed by pathology as Wegener’s Granulomatosis was ruled out.

Classification

In contrast with B-cell lymphoma, the classification of such a rare neoplasm has been controversial, since the cytologic features have not been very useful. Further, by many entities, T-cell and natural killer cell (NK) neoplasms do not share any similar immuno-phenotype. Because of such matters, clinical features became handier for classification and somehow even more important than the precise cell of origin. since the majority of cytotoxic T-cell and NK cell lymphomas are located out of lymph nodes, the gene expressing cytotoxic molecules may predispose to apoptosis by tumor cells and by standard cells.Three major categories of extranodal T/NK cell tumors include: Extranodal NK/T-cell lymphoma, nasal type lymphoma, Extranodal NK/T-cell lymphoma, enteropathy type lymphoma, Extranodal NK/T-cell lymphoma, subcutaneous panniculitis-like, Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes:, NK cell-derived neoplasms, namely, aggressive NK cell leukemia, Extranodal NK/T-cell lymphoma, nasal type, Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into: Extranodal NK-T-cell lymphoma, nasal type, Extranodal NK-T-cell lymphoma, extra nasal type

Pathophysiology

NK cells are CD3 and myloperoxidase negative on their surface. NK cells have germline configuration of T-cell receptor and immunoglobulin genes. NK cells originate from a bipotent NK/T-progenitor cell so they have a lot in common with T cells. NK cells express T-associate markers such as CD2, CD3e, CD7, CD8, CD16, CD56, CD57. Most tumors involve NK cells but also involve T cells as neoplastic cells, that is the main reason of classification as NK/T-cell lymphoma rather than NK-cell lymphoma. the immunophenotype of NK lymphoma cells is classically positive for CD2, CD56, and cytoplasmic CD3 epsilon. they are negative for surface CD3. Unlike normal NK cells, the tumor cells are usually negative for CD7 and CD16. they express cytotoxic granule associated proteins granzyme B, T-cell restricted intracellular antigen (TIA-1), and perforin. NK/T cell lymphoma expressing CD56 is rare but most commonly occurs int he head and neck region, skin, and soft tissue. genes involved in the pathogenesis of extranodal NK-T-cell lymphoma include.:HLA DQA1*0501, HLA DQB1*0201. these genes also are relevant with celiac disease.On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma.Coagulative necrosis and apoptotic bodies are frequently encountered., Extranodal NK/T-cell lymphoma, nasal type occurs in nasal cavity and upper aerodigestive tract., Extranodal NK/T cell lymphoma, nasal type affects the nose and facial mid line exhibiting aggressive destruction., on microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma. the tumor cells are small to medium in size with occasional large and anaplastic forms. the lymphoma cells may be admixed with a polymorphic infiltrate of nonneoplastic inflammatory cells including small lymphocytes, plasma cells, histiocytes, and eosinophils.

Causes

Extranodal NK/T-cell lymphoma is caused by transition mutation of p53. P53 overexpresion has assosiation with poor prognosis.

Differential Diagnosis

Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as NK cell lukemia, lymphomatoid granulomatosis, EBV-positive diffuse large B cell lymphoma, NOS, anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, peripheral T cell lymphoma, hepatosplenic T cell lymphoma. Extranodal NK/T-cell lymphoma, nasal type must be diffrentiated from Wegner’s granulomatosis, Polymorphic reticulosis, and Midline malignant lymphoma

Epidemiology and Demographics

NK cell lymphoma shows a poor prognosis because of rapid local progression and distant metastasis.The median age of onset is approximately 50 years and it is common in elderly. extranodal NK/T-cell lymphoma is a rare disease in children and often it is associated with mosquito-bite hypersensitivity or other EBV-associated disease.Natural Killer (NK) cell lymphoma is a rare disease. extranodal NK/T cell lymphoma, nasal type (NKTCL) and aggressive NK-cell leukemia (ANKCL) have a higher incidence in Asia, Central, and South America.NK T cell lymphoma, nasal type (NNKTL) consist 3000-10000 out of 100000 cases of non-Hodgkin lymphoma in Asia and South America and less than 1000 in 10000 patient in western countries.

Risk Factors

There is a strong association between with extranodal NK/T-cell lymphoma and EBV virus.There is a strong assosiation between Extranodal NK/T-cell lymphoma andFas(Po-1/CD95) mutations.

Screening

According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.

Natural History, Prognosis, and Complications

If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.

Diagnosis

Extranodal NK/T cell lymphoma survival rate is not well predicted by Ann Arbor staging system. A new system was introduced based on B symptoms, Ann Arbor system, LDH level, and regional lymphadenopathy combined. Another parallel mechanism for extranodal NK/T cell lymphoma is based on tumor biologic and micro-environmental factors. High Ki-67 nuclear antigen is a marker for actively proliferation tumor cell which could be a related factor with mass bulk.

combination of these two findings on immunophenotyping of frozen section specimen cells and RT-PCR are confirmatory for Extranodal NK/T cell lymphoma:Detection of CD56 and EBER1 on tumor cells and EBV DNA (RT-PCR).Pathological examination of frozen section specimen may misled the NK/T cell diagnosis.Circulating cell free EBV DNA levels by RT-PCR is a diagnostic factor for EBV associated malignancies, Like extranodal NK/T cell lymphoma. Measuring both BamHI W DNA and LMP1 DNA is more useful as a prognosis predictor, these DNAs will decrease with treatment and increase with relapse. Recently, determination of negative immune checkpoints are considered as a drug target,in the case of extranodal NK/T cell lymphoma, PD-L1 will be expressed in the tumor, and patients with higher concentration of soluble PD-L1 showed worse prognosis.

The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Extranodal NK/T cell lymphoma, nasal type, which is the most common sub type, is usually found as an ulcerative and necrotic granuloma in the nasal cavity, palate, and nasopharynx. Tumor can spread to surrounding tissue such as facial skin, paranasal sinus, and orbits, and cause exensive destruction of midline lesions. The most common symptoms at the time of diagnosis are nasal obstruction and bloody rhinorea.

Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.

Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.

X-rays graphy of patients with extranodal NK/T cell lymphoma is not very usefull in order of diagnosis. Radiography show non-specific tumor changes.

CT scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma. Computerized tomography (CT) may be performed to detect metastasis and assess the local extent of extranodal NK-T-cell lymphoma.

MRI may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.Magnetic resonance imaging (MRI) may be performed to better define local soft tissue and bony involvement of extranodal NK-T-cell lymphoma.

Treatment

The predominant therapy for extranodal NK-T-cell lymphoma is radiation therapy. Adjudicative chemotherapy and stem cell transplant may be required.

Surgical intervention is not recommended for the management of extranodal NK/T cell lymphoma.

There are no established measures for the primary prevention of extranodal NK/T cell lymphoma.

Effective measures for the secondary prevention of extranodal NK-T-cell lymphoma include: LDH , MLH1, PDGFRA, VEGF, PD-L1, PD-1, Cyclin D1, p53, Ki-67.

High dose chemotherapy and autologous haematopoietic stem cell transplantation is used for varying types of lymphoma, however there is a lac of such experience in treating extranodal NK-T cell lymphoma. Further clinical trials are needed to prove that this therapy is handy and can be used as a method of therapy. Autologous transplantation appears to provide a great survival benefit only for those who attended a complete remission at the time of transplantation.

References

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

Extranodal NK cell lymphoma probably was first reported by McBride as a disease which rapidly destructs nose and face with progressing necrotic granuloma. The natural history of extranodal NK cell lymphoma was generally aggressive and lethal, this disease was initially termed as “rhinitis gangrenosa progressiva”. Since the lesions usually was in midline and was aggressive and lethal, the term “lethal midline granuloma (LMG)” was used. First known case of natural-killer-cell lymphoma was diagnosed in a 19-years old boy. The diagnosis of natural-killer-cell lymphoma was confirmed by pathology as Wegener’s Granulomatosis was ruled out.

Historical Perspective

Discovery

  • Extranodal NK cell lymphoma probably was first scovered by McBride as a disease which rapidly destructs nose and face with progressing necrotic granuloma.[1]
  • First known case of natural-killer-cell lymphoma was a 19-years old man, with a tumor affecting nasal cavity.[2]
  • Natural-Killer-cell lymphoma was diagnosed as “Granuloma Gangraenescens” which was considered as unclassified sarcoma.
  • In 1988, 33 years after the first confirmed diagnosis of NK cell lymphoma, the patient had a relapse with skin lesions which progressed with nasal involvement in 2002, and the final mortal relapse was in 2003 with the leukemic disease.[3]
  • Reanalysis of stored slides of 1965 revealed an immunophenotype typical of NK cell which was similar to those found in 1998 skin lesions, 2002 nasal tissue, and 2003 blood.
  • Biopsy specimen collected in 1998 and 2002 contained EBV virus encoded RNA transcripts and then diagnosed as a natural-killer-cell lymphoma.

Landmark Events in the Development of Treatment Strategies

  • The natural history of extranodal NK cell lymphoma was generally aggressive and lethal, this disease was initially termed as “rhinitis gangrenosa progressiva”.[4]
  • Since the lesions usually was in midline and was aggressive and lethal, the term “lethal midline granuloma (LMG)” was used.[5]
  • The diagnosis of natural-killer-cell lymphoma was confirmed by pathology as Wegener’s Granulomatosis was ruled out.


References

  1. ↑ Harabuchi Y, Takahara M, Kishibe K, Nagato T, Kumai T (2019). “Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type: Basic Science and Clinical Progress”. Front Pediatr. 7: 141. doi:10.3389/fped.2019.00141. PMC 6476925. PMID 31041299.
  2. ↑ Schrader C, Janssen D, Kneba M, Lennert K (2004). “A 38-year history of natural-killer-cell lymphoma”. N Engl J Med. 350 (4): 418–9. doi:10.1056/NEJM200401223500423. PMID 14736941.
  3. ↑ Sawada, Akihisa; Inoue, Masami (2018). “Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders”. Frontiers in Pediatrics. 6. doi:10.3389/fped.2018.00334. ISSN 2296-2360.
  4. ↑ McBride P (1991). “Photographs of a case of rapid destruction of the nose and face. 1897”. J Laryngol Otol. 105 (12): 1120. doi:10.1017/s0022215100118407. PMID 1787378.
  5. ↑ Aozasa, Katsuyuki; Takakuwa, Tetsuya; Hongyo, Tadashi; Yang, Woo-Ick (2008). “Nasal NK/T-cell lymphoma: epidemiology and pathogenesis”. International Journal of Hematology. 87 (2): 110–117. doi:10.1007/s12185-008-0021-7. ISSN 0925-5710.


Template:WikiDoc Sources

Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

In contrast with B-cell lymphoma, the classification of such a rare neoplasm has been controversial, since the cytologic features have not been very useful. Further, by many entities, T-cell and natural killer cell (NK) neoplasms do not share any similar immuno-phenotype. Because of such matters, clinical features became handier for classification and somehow even more important than the precise cell of origin. since the majority of cytotoxic T-cell and NK cell lymphomas are located out of lymph nodes, the gene expressing cytotoxic molecules may predispose to apoptosis by tumor cells and by standard cells.Three major categories of extranodal T/NK cell tumors include: Extranodal NK/T-cell lymphoma, nasal type lymphoma, Extranodal NK/T-cell lymphoma, enteropathy type lymphoma, Extranodal NK/T-cell lymphoma, subcutaneous panniculitis-like, Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes:, NK cell-derived neoplasms, namely, aggressive NK cell leukemia, Extranodal NK/T-cell lymphoma, nasal type, Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into: Extranodal NK-T-cell lymphoma, nasal type, Extranodal NK-T-cell lymphoma, extra nasal type


Classification

  • In contrast with B-cell lymphoma, the classification of such a rare neoplasm has been controversial, since the cytologic features have not been very useful. Further, by many entities, T-cell and natural killer cell (NK) neoplasms do not share any similar immuno-phenotype. Because of such matter, clinical features became handier for classification and somehow even more important than the precise cell of origin.[1][2]
Extranodal NK-T-cell lymphoma classification
Name Description
Extranodal NK-T-cell lymphoma, nasal type
Extranodal NK-T-cell lymphoma, extra nasal type
  • Can affect the:

Natural Killer cell neoplasms are classified by the world health organization (WHO) into:

  • NK/T-cell lymphoma, nasal type (NKTCL) [5]
  • Aggressive NK-cell leukemia (ANKCL)
  • Chronic lymphoproliferative disorders of NK-cell

References

  1. ↑ 1.0 1.1 Extranodal natural killer/T-cell lymphoma. BioMedCentral. http://jhoonline.biomedcentral.com/articles/10.1186/1756-8722-6-86. Accessed on February 19, 2016
  2. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016,
  3. ↑ Jaffe ES, Krenacs L, Raffeld M (2003). “Classification of cytotoxic T-cell and natural killer cell lymphomas”. Semin Hematol. 40 (3): 175–84. PMID 12876666.
  4. ↑ Extranodal NK/T-cell lymphoma, nasal type. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/extranodal-nk-t-cell-lymphoma-nasal-type/?region=on. Accessed on February 18, 2016
  5. ↑ Lima M (2013). “Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis”. Orphanet J Rare Dis. 8: 95. doi:10.1186/1750-1172-8-95. PMC 3770456. PMID 23816348.


Template:WikiDoc Sources

Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

NK cells are CD3 and myloperoxidase negative on their surface. NK cells have germline configuration of T-cell receptor and immunoglobulin genes. NK cells originate from a bipotent NK/T-progenitor cell so they have a lot in common with T cells. NK cells express T-associate markers such as CD2, CD3e, CD7, CD8, CD16, CD56, CD57. Most tumors involve NK cells but also involve T cells as neoplastic cells, that is the main reason of classification as NK/T-cell lymphoma rather than NK-cell lymphoma. the immunophenotype of NK lymphoma cells is classically positive for CD2, CD56, and cytoplasmic CD3 epsilon. they are negative for surface CD3. Unlike normal NK cells, the tumor cells are usually negative for CD7 and CD16. they express cytotoxic granule associated proteins granzyme B, T-cell restricted intracellular antigen (TIA-1), and perforin. NK/T cell lymphoma expressing CD56 is rare but most commonly occurs int he head and neck region, skin, and soft tissue. genes involved in the pathogenesis of extranodal NK-T-cell lymphoma include.:HLA DQA1*0501, HLA DQB1*0201. these genes also are relevant with celiac disease.On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma.Coagulative necrosis and apoptotic bodies are frequently encountered., Extranodal NK/T-cell lymphoma, nasal type occurs in nasal cavity and upper aerodigestive tract., Extranodal NK/T cell lymphoma, nasal type affects the nose and facial mid line exhibiting aggressive destruction., on microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma. the tumor cells are small to medium in size with occasional large and anaplastic forms. the lymphoma cells may be admixed with a polymorphic infiltrate of nonneoplastic inflammatory cells including small lymphocytes, plasma cells, histiocytes, and eosinophils.

Pathophysiology

Physiology

  • NK cells are CD3 and myloperoxidase negative on their surface.[1]
  • NK cells have germline configuration of T-cell receptor and immunoglobulin genes.[2]
  • NK cells originate from a bipotent NK/T-progenitor cell so they have a lot in common with T cells.[3]
  • NK cells express T-associate markers such as CD2, CD3e, CD7, CD8, CD16, CD56, CD57.[4]
  • Most tumors involve NK cells but also involve T cells as neoplastic cells, that is the main reason of classification as NK/T-cell lymphoma rather than NK-cell lymphoma.[5]

Immunotype

  • The immunophenotype of NK lymphoma cells is classically positive for CD2, CD56, and cytoplasmic CD3 epsilon.[6]
  • They are negative for surface CD3. Unlike normal NK cells, the tumor cells are usually negative for CD7 and CD16.
  • They express cytotoxic granule associated proteins granzyme B, T-cell restricted intracellular antigen (TIA-1), and perforin.
  • NK/T cell lymphoma expressing CD56 is rare but most commonly occurs int he head and neck region, skin, and soft tissue.

Genetics

Genes involved in the pathogenesis of extranodal NK-T-cell lymphoma include.[7]:

These genes also are relevant with celiac disease.

Gross Pathology

Adopted from OPEN-I. Nasal extranodal natural killer cell/T cell lymphoma with destruction of the midline[9]

Microscopic Pathology

  • On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.[10]
  • The tumor cells are small to medium in size with occasional large and anaplastic forms.
  • The lymphoma cells may be admixed with a polymorphic infiltrate of nonneoplastic inflammatory cells including small lymphocytes, plasma cells, histiocytes, and eosinophils.
Primary extranodal NK/T cell lymphoma, nasal-type[11]

References

  1. ↑ Ham, Maria Francisca; Ko, Young-Hyeh (2010). “Natural killer cell neoplasm: biology and pathology”. International Journal of Hematology. 92 (5): 681–689. doi:10.1007/s12185-010-0738-y. ISSN 0925-5710.
  2. ↑ Extranodal NK/T-cell lymphoma, nasal type. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/extranodal-nk-t-cell-lymphoma-nasal-type/?region=on. Accessed on February 19, 2016
  3. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016
  4. ↑ Stewart CA, Walzer T, Robbins SH, Malissen B, Vivier E, Prinz I (2007). “Germ-line and rearranged Tcrd transcription distinguish bona fide NK cells and NK-like gammadelta T cells”. Eur J Immunol. 37 (6): 1442–52. doi:10.1002/eji.200737354. PMID 17492716.
  5. ↑ Jaffe ES, Nicolae A, Pittaluga S (January 2013). “Peripheral T-cell and NK-cell lymphomas in the WHO classification: pearls and pitfalls”. Mod. Pathol. 26 Suppl 1: S71–87. doi:10.1038/modpathol.2012.181. PMC 6324567. PMID 23281437.
  6. ↑ Ko, Y H; Cho, E-Y; Kim, J-E; Lee, S-S; Huh, J-R; Chang, H-K; Yang, W-I; Kim, C-W; Kim, S-W; Ree, H J (2004). “NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status”. Histopathology. 44 (5): 480–489. doi:10.1111/j.1365-2559.2004.01867.x. ISSN 0309-0167.
  7. ↑ Swerdlow, Steven (2017). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer. ISBN 9789283244943.
  8. ↑ Ko, Y H; Cho, E-Y; Kim, J-E; Lee, S-S; Huh, J-R; Chang, H-K; Yang, W-I; Kim, C-W; Kim, S-W; Ree, H J (2004). “NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status”. Histopathology. 44 (5): 480–489. doi:10.1111/j.1365-2559.2004.01867.x. ISSN 0309-0167.
  9. ↑ “GMS | GMS Current Topics in Otorhinolaryngology – Head and Neck Surgery | Orphan diseases of the nose and paranasal sinuses: Pathogenesis – clinic – therapy”.
  10. ↑ Liu, Chih-Yi; Tsai, Hui-Chih (2018). “Comparison of Diagnostic Cytomorphology of Natural Killer/T-Cell Lymphoma (Nasal Type) in Conventional Smears, Liquid-Based Preparations, and Histopathology”. Case Reports in Pathology. 2018: 1–7. doi:10.1155/2018/6264810. ISSN 2090-6781.
  11. ↑ Fang, Jian-chen; Zhou, Jue; Li, Zheng; Xia, Zhao-xia (2014). “Primary extranodal NK/T cell lymphoma, nasal-type of uterus with adenomyosis: a case report”. Diagnostic Pathology. 9 (1). doi:10.1186/1746-1596-9-95. ISSN 1746-1596.


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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

Extranodal NK/T-cell lymphoma is caused by transition mutation of p53. P53 overexpresion has assosiation with poor prognosis.

Causes

Common Causes

Common causes of extranodal NK-T cell lymphoma may include:[1]

  • transition mutation of p53.
  • P53 overexertion association with poor prognosis.

Genetic causes:

  • Extranodal NK/T-cell lymphoma is caused by transition mutation of p53.[2]
  • Genetic instability of micro satellites was not found in cases with the disease.[3]
  • P53 overexertion has association with poor prognosis.

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect No underlying causes
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic No underlying causes
Hematologic No underlying causes
Iatrogenic No underlying causes
Infectious Disease EBV infection
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic P53 mutation
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy No underlying causes
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes


Causes in Alphabetical Order

List the causes of the disease in alphabetical order:

  • EBV infection
  • P53 gene mutation

References

  1. ↑ Harabuchi, Yasuaki; Takahara, Miki; Kishibe, Kan; Nagato, Toshihiro; Kumai, Takumi (2019). “Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type: Basic Science and Clinical Progress”. Frontiers in Pediatrics. 7. doi:10.3389/fped.2019.00141. ISSN 2296-2360.
  2. ↑ Aozasa, Katsuyuki; Takakuwa, Tetsuya; Hongyo, Tadashi; Yang, Woo-Ick (2008). “Nasal NK/T-cell lymphoma: epidemiology and pathogenesis”. International Journal of Hematology. 87 (2): 110–117. doi:10.1007/s12185-008-0021-7. ISSN 0925-5710.
  3. ↑ Greenblatt MS, Bennett WP, Hollstein M, Harris CC (1994). “Mutations in the p53 tumor suppressor gene: clues to cancer etiology and molecular pathogenesis”. Cancer Res. 54 (18): 4855–78. PMID 8069852.


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Differentiating Extranodal NK-T-cell lymphoma from other Diseases

Differentiating Extranodal NK-T-cell lymphoma from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

Overview

Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as NK cell lukemia, lymphomatoid granulomatosis, EBV-positive diffuse large B cell lymphoma, NOS, anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, peripheral T cell lymphoma, hepatosplenic T cell lymphoma

Extranodal NK/T-cell lymphoma, nasal type must be diffrentiated from Wegner’s granulomatosis, Polymorphic reticulosis, and Midline malignant lymphoma

Differential Diagnosis

  • Extranodal NK t-cell must be differentiated with other NK and T cell hematologic malignancies and EBV-associated T cell or NK cell lymphoprolifrative disorders.[1]

Differentiating extranodal NK T-cell from other hematologic malignancies by immunophenotype and EBV infection:

Extranodal NK T-cell lymphoma express CD56 on its surface accompanied with EBV infection.

Malignancies based on immunophenotype and EBV infection
Disease Immunophenotype EBV
NK cell leukemia CD16+ +
Lymphomatoid granulomatosis CD20+ +
EBV-positive diffuse large B cell lymphoma, NOS CD20+ CD76+ +
Peripheral T cell lymphoma, unspecified CD3+ CD56– –
Anaplastic large cell lymphoma CD30+ –
Hepatosplenic T cell lymphoma CD2+ CD7+ CD3+ –

References

  1. ↑ Karube, Kennosuke; Aoki, Ryosuke; Nomura, Yuko; Yamamoto, Kohei; Shimizu, Kay; Yoshida, Shirou; Komatani, Hideki; Sugita, Yasuo; Ohshima, Koichi (2008). “Usefulness of flow cytometry for differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas: Analysis of 490 cases”. Pathology International. 58 (2): 89–97. doi:10.1111/j.1440-1827.2007.02195.x. ISSN 1320-5463.
  2. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016
  3. ↑ Kassel SH, Echevarria RA, Guzzo FP (1969). “Midline malignant reticulosis (so-called lethal midline granuloma)”. Cancer. 23 (4): 920–35. doi:10.1002/1097-0142(196904)23:4<920::aid-cncr2820230430>3.0.co;2-m. PMID 5818523.
  4. ↑ Kassel SH, Echevarria RA, Guzzo FP (1969). “Midline malignant reticulosis (so-called lethal midline granuloma)”. Cancer. 23 (4): 920–35. doi:10.1002/1097-0142(196904)23:4<920::aid-cncr2820230430>3.0.co;2-m. PMID 5818523.


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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

Overview

NK cell lymphoma shows a poor prognosis because of rapid local progression and distant metastasis.The median age of onset is approximately 50 years and it is common in elderly. extranodal NK/T-cell lymphoma is a rare disease in children and often it is associated with mosquito-bite hypersensitivity or other EBV-associated disease.Natural Killer (NK) cell lymphoma is a rare disease. extranodal NK/T cell lymphoma, nasal type (NKTCL) and aggressive NK-cell leukemia (ANKCL) have a higher incidence in Asia, Central, and South America.NK T cell lymphoma, nasal type (NNKTL) consist 3000-10000 out of 100000 cases of non-Hodgkin lymphoma in Asia and South America and less than 1000 in 10000 patient in western countries

Epidemiology and Demographics

Mortality rate

Age


Race

  • Natural Killer (NK) cell lymphoma is a rare disease. NK/T cell lymphoma, nasal type (NKTCL) and aggressive NK-cell leukemia (ANKCL) have a higher incidence in Asia, Central, and South America.
  • Nk cell lymphoma is usually associated with Epstein-Barr virus infection.[3]


Gender


Region

  • Extranodal NK T cell lymphoma, nasal type (NNKTL) consist 3000-10000 out of 100000 cases of non-Hodgkin lymphoma in Asia and South America and less than 1000 in 10000 patient in western countries.
  • It is estimated the incidence of NNKTL is higher in Asia by 10-folds.[5]


References

  1. ↑ Jaffe, Elaine S; Nicolae, Alina; Pittaluga, Stefania (2013). “Peripheral T-cell and NK-cell lymphomas in the WHO classification: pearls and pitfalls”. Modern Pathology. 26 (S1): S71–S87. doi:10.1038/modpathol.2012.181. ISSN 0893-3952.
  2. ↑ Harabuchi, Yasuaki; Takahara, Miki; Kishibe, Kan; Nagato, Toshihiro; Kumai, Takumi (2019). “Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type: Basic Science and Clinical Progress”. Frontiers in Pediatrics. 7. doi:10.3389/fped.2019.00141. ISSN 2296-2360.
  3. ↑ Lima, Margarida (2013). “Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis”. Orphanet Journal of Rare Diseases. 8 (1): 95. doi:10.1186/1750-1172-8-95. ISSN 1750-1172.
  4. ↑ Wang, Xiaotian; Gong, Zimu; Li, Shawn Xiang; Yan, Wei; Song, Yongsheng (2017). “Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature”. BMC Urology. 17 (1). doi:10.1186/s12894-017-0273-8. ISSN 1471-2490.
  5. ↑ Haverkos, Bradley M.; Pan, Zenggang; Gru, Alejandro A.; Freud, Aharon G.; Rabinovitch, Rachel; Xu-Welliver, Meng; Otto, Brad; Barrionuevo, Carlos; Baiocchi, Robert A.; Rochford, Rosemary; Porcu, Pierluigi (2016). “Extranodal NK/T Cell Lymphoma, Nasal Type (ENKTL-NT): An Update on Epidemiology, Clinical Presentation, and Natural History in North American and European Cases”. Current Hematologic Malignancy Reports. 11 (6): 514–527. doi:10.1007/s11899-016-0355-9. ISSN 1558-8211.
Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2]

Overview

There is a strong association between with extranodal NK/T-cell lymphoma and EBV virus.There is a strong assosiation between Extranodal NK/T-cell lymphoma andFas(Po-1/CD95) mutations.

Risk Factors

  • There is a strong association between with extranodal NK/T-cell lymphoma and EBV virus.[1]
  • Studies revealed that there is a constant assosiation between NK/T-cell lymphoma with EBV in the world.[2][3][4]
  • There is a strong assosiation between Extranodal NK/T-cell lymphoma andFas(Po-1/CD95) mutations.[5]

References

  1. ↑ Ng SB, Khoury JD (2009). “Epstein-Barr virus in lymphoproliferative processes: an update for the diagnostic pathologist”. Adv Anat Pathol. 16 (1): 40–55. doi:10.1097/PAP.0b013e3181916029. PMID 19098466.
  2. ↑ Kanavaros P, Lescs MC, Brière J, Divine M, Galateau F, Joab I; et al. (1993). “Nasal T-cell lymphoma: a clinicopathologic entity associated with peculiar phenotype and with Epstein-Barr virus”. Blood. 81 (10): 2688–95. PMID 8387835.
  3. ↑ Mishima, Kenji; Horiuchi, Keisuke; Kojya, Shizuo; Takahashi, Hiroomi; Ohsawa, Masahiko; Aozasa, Katsuyuki (1994). “Epstein-barr virus in patients with polymorphic reticulosis (lethal midline granuloma) from china and japan”. Cancer. 73 (12): 3041–3046. doi:10.1002/1097-0142(19940615)73:12<3041::AID-CNCR2820731224>3.0.CO;2-K. ISSN 0008-543X.
  4. ↑ Harabuchi Y, Yamanaka N, Kataura A, Imai S, Kinoshita T, Mizuno F; et al. (1990). “Epstein-Barr virus in nasal T-cell lymphomas in patients with lethal midline granuloma”. Lancet. 335 (8682): 128–30. doi:10.1016/0140-6736(90)90002-m. PMID 1967431.
  5. ↑ Suda T, Takahashi T, Golstein P, Nagata S (1993). “Molecular cloning and expression of the Fas ligand, a novel member of the tumor necrosis factor family”. Cell. 75 (6): 1169–78. doi:10.1016/0092-8674(93)90326-l. PMID 7505205.


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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

Overview

According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.

Screening

According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.[1]

References

  1. ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=extranodal+NK%2FT+cell+lymphoma. Accessed on February 02, 2016


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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

Overview

If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.

Natural History

  • Extranodal NK-T-cell lymphoma almost always shows an extranodal presentation.[1][2]
  • Some cases may be accompanied by secondary lymph node involvement although rare instances of primary lymph node disease in the absence of extranodal involvement has been reported.
  • Extension to adjacent tissues such as the nasopharynx, paranasal sinuses, orbit, oral cavity, palate, and oropharynx are possible.
  • Retro-orbital involvement causes proptosis and impairment of extraocular movement.
  • Occasionally local extension from the nasal cavity causes destruction of the hard palate with the characteristic midline perforation, previously referred to as “lethal midline granuloma”.
  • At presentation, the disease is often localized to the upper aerodigestive tract.
  • Disseminated disease may have involvement of the lymph nodes, bone marrow and peripheral blood.

Complications

  • Extranodal NK-T-cell lymphoma, nasal type, often develop hemophagocytic syndrome. (uncontrolled activation of certain parts of the immune system)[3]
  • Hemophagocytic syndrome results in

Prognosis

  • The prognosis for people with extranodal NK-T-cell lymphoma is often poor and the risk for relapse is high.[3]
  • People with extranodal NK-T-cell lymphoma confined to the nose or nasal passages have a better prognosis than those people with more widespread disease.
  • The International Prognostic Index (IPI) predicts outcome in nasal NK-T-cell lymphoma.[4]
  • Patients with International Prognostic Index of 1 or less were shown to have a better overall survival.
  • In a recent retrospective analysis of 172 patients with extranodal NK-T-cell lymphoma, nasal type 4 prognostic factors were identified :
  • Nonnasal type
  • Stage
  • Performance status
  • Number of extranodal sites
  • In a Korean prognostic model, four prognostic groups have been identified depending on :

References

  1. ↑ Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016
  2. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016
  3. ↑ 3.0 3.1 Extranodal NK/T-cell lymphoma, nasal type. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/extranodal-nk-t-cell-lymphoma-nasal-type/?region=on. Accessed on February 04, 2016
  4. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 04, 2016


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Diagnosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies


Treatment

Treatment

Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1



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