Extranodal NK-T-cell lymphoma pathophysiology

Jump to navigation Jump to search

Extranodal NK-T-cell lymphoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Extranodal NK-T-cell lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Xray

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Extranodal NK-T-cell lymphoma pathophysiology On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Extranodal NK-T-cell lymphoma pathophysiology

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Extranodal NK-T-cell lymphoma pathophysiology

CDC on Extranodal NK-T-cell lymphoma pathophysiology

Extranodal NK-T-cell lymphoma pathophysiology in the news

Blogs on Extranodal NK-T-cell lymphoma pathophysiology

Risk calculators and risk factors for Extranodal NK-T-cell lymphoma pathophysiology

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

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.Natural killer cells are a type of lymphocyte that is closely related to T cells and attack foreign cells. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium-sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.[1][2]

Pathophysiology

Physiology

  • NK cells are CD3 and myloperoxidase negative on their surface.[3]
  • 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.[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.

Immunotype

  • 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.[5]

Gross Pathology

  • 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.
Adopted from OPEN-I. Nasal extranodal natural killer cell/T cell lymphoma with destruction of the midline[6]

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.
  • 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[7]

References

  1. Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016
  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. 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.
  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. 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.
  6. . doi:10.3205/cto000119. Missing or empty |title= (help)
  7. 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.


Template:WikiDoc Sources