Aggressive NK-cell leukemia: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
|||
| Line 7: | Line 7: | ||
==Overview== | ==Overview== | ||
Aggressive NK-cell leukemia is a [[disease]] with an aggressive, systemic proliferation of [[natural killer cells|natural killer cells (NK cells)]] and a rapidly declining clinical course. There is no [[classification]] system for aggressive NK-cell leukemia. The [[pathogenesis]] of aggressive NK-cell leukemia is characterized by the proliferation of [[Natural killer cell|natural killer cells]]. Aggressive NK-cell leukemia is very uncommon, and is most commonly seen among middle aged adults. The most common [[Risk factor|risk factors]] in the development of aggressive NK-cell leukemia is the [[Epstein-Barr virus|Epstein-Barr virus (EBV)]] [[infection]]. The majority of [[Patient|patients]] with aggressive NK-cell leukemia are [[symptomatic]] at [[diagnosis]]. Early clinical features include [[fatigue]], [[Night sweat|night sweats]], and [[fever]]. [[Physical examination]] among [[Patient|patients]] with aggressive NK-cell leukemia may be remarkable for [[hepatosplenomegaly]]. Aggressive NK-cell leukemia may also be [[Diagnosis|diagnosed]] using [[bone marrow biopsy]], findings of which can include extensive [[marrow]] replacement by [[Leukemia|leukemic]] [[Cell (biology)|cells]] and reactive [[Histiocyte|histiocytes]] displaying [[hemophagocytosis]]. The mainstay of [[therapy]] for aggressive NK-cell leukemia is [[anthracycline]]-containing [[chemotherapy]]. | |||
and reactive histiocytes displaying hemophagocytosis. The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy. | |||
== Classification== | == Classification== | ||
There is no [[classification]] system for aggressive NK-cell leukemia.<ref name="cha2">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10389582&query_hl=10&itool=pubmed_ExternalLink] | |||
Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | ||
==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of aggressive NK-cell leukemia is characterized by the aggressive, systemic proliferation of natural killer cells. | *The [[pathogenesis]] of aggressive NK-cell leukemia is characterized by the aggressive, systemic proliferation of [[Natural killer cell|natural killer cells]]. | ||
*The mutation in oncogene p53 has been associated with the development of aggressive NK-cell leukemia. | *The [[mutation]] in [[oncogene]] [[p53]] has been associated with the development of aggressive NK-cell leukemia. | ||
*On gross pathology, characteristic findings of aggressive NK-cell leukemia | *On [[gross pathology]], characteristic findings of aggressive NK-cell leukemia include: | ||
:*No remarkable findings | :*No remarkable findings | ||
*On microscopic histopathological analysis, characteristic findings of aggressive NK-cell leukemia | *On [[microscopic]] [[Histopathology|histopathological]] analysis, characteristic findings of aggressive NK-cell leukemia include:<ref name="cha2">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10389582&query_hl=10&itool=pubmed_ExternalLink] | ||
Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | ||
:*Large cells with abundant blue cytoplasm | |||
:*Azurophilic granules | :*Large [[Cell (biology)|cells]] with abundant blue [[cytoplasm]] | ||
:*Irregular nuclei | :*[[Azurophilic granules]] | ||
:*Open chromatin | :*Irregular [[Cell nucleus|nuclei]] | ||
:*Distinct nucleoli | :*Open [[chromatin]] | ||
:*CD11b and CD16 show variable expression | :*Distinct [[Nucleolus|nucleoli]] | ||
*The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia.<ref name="cha2">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10389582&query_hl=10&itool=pubmed_ExternalLink] | :*[[CD11b]] and [[CD16]] show variable expression | ||
*The table below demonstrates the [[Immunophenotyping|immunophenotype]] for [[Patient|patients]] with aggressive NK-cell leukemia.<ref name="cha2">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10389582&query_hl=10&itool=pubmed_ExternalLink] | |||
Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | Chan JK. "Natural killer cell neoplasms." '''Anat Pathol'''. 1998;3:77-145. PMID 10389582</ref> | ||
::{| border="1" cellpadding="5" cellspacing="0" | ::{| border="1" cellpadding="5" cellspacing="0" | ||
|- | |- | ||
| Line 41: | Line 39: | ||
|- | |- | ||
| rowspan="1" | Negative | | rowspan="1" | Negative | ||
| colspan="1" align="center" | [[CD57]] | | colspan="1" align="center" |[[CD57]] | ||
|- | |- | ||
|} | |} | ||
Revision as of 20:41, 17 October 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [3]
Synonyms and keywords: ANKL
Overview
Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells (NK cells) and a rapidly declining clinical course. There is no classification system for aggressive NK-cell leukemia. The pathogenesis of aggressive NK-cell leukemia is characterized by the proliferation of natural killer cells. Aggressive NK-cell leukemia is very uncommon, and is most commonly seen among middle aged adults. The most common risk factors in the development of aggressive NK-cell leukemia is the Epstein-Barr virus (EBV) infection. The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis. Early clinical features include fatigue, night sweats, and fever. Physical examination among patients with aggressive NK-cell leukemia may be remarkable for hepatosplenomegaly. Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy, findings of which can include extensive marrow replacement by leukemic cells and reactive histiocytes displaying hemophagocytosis. The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.
Classification
There is no classification system for aggressive NK-cell leukemia.[1]
Pathophysiology
- The pathogenesis of aggressive NK-cell leukemia is characterized by the aggressive, systemic proliferation of natural killer cells.
- The mutation in oncogene p53 has been associated with the development of aggressive NK-cell leukemia.
- On gross pathology, characteristic findings of aggressive NK-cell leukemia include:
- No remarkable findings
- On microscopic histopathological analysis, characteristic findings of aggressive NK-cell leukemia include:[1]
- The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia.[1]
Causes
- There are no established causes for aggressive NK-cell leukemia.
Differentiating Aggressive NK-cell Leukemia from Other Diseases
- Aggressive NK-cell leukemia must be differentiated from other diseases that cause fever, fatigue, and lymphadenopathy such as:
- Human immunodeficiency virus
- Chronic neutrophilic leukemia
- Juvenile myelomonocytic leukemia
Epidemiology and Demographics
- Aggressive NK-cell leukemia is very uncommon.[2]
Age
- Aggressive NK-cell leukemia is more commonly observed among middle aged adults.
Gender
- Aggressive NK-cell leukemia affects men and women equally.
Race
- Aggressive NK-cell leukemia usually affects individuals of the Asian race.
Risk Factors
- Common risk factors in the development of aggressive NK-cell leukemia, include:
- Epstein-Barr virus (EBV) infection
Natural History, Complications and Prognosis
- The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis.[2]
- Early clinical features include fatigue, night sweats, and fever.
- If left untreated, patients with aggressive NK-cell leukemia may progress to develop recurrent infections.
- Common complications of aggressive NK-cell leukemia, include:
- Coagulopathies
- Hemophagocytic syndrome
- Multiple organ failure
- Myelofibrosis
- Prognosis is generally poor, and the median survival rate of patients with aggressive NK-cell leukemia is approximately 12 months.[2]
Diagnosis
Symptoms
- Aggressive NK-cell leukemia is usually asymptomatic.
- Symptoms of aggressive NK-cell leukemia may include the following:[1]
- Fever
- Swelling in the lymph nodes in the neck
- Night sweats
- Persistent fatigue
- Loss of appetite
- Weight loss
- Nausea
- Vomiting
- Paleness
- Bleeding from the gums
- Enlarged liver or spleen
Physical Examination
- Patients with aggressive NK-cell leukemia usually appear pale and malnourished.
- Physical examination may be remarkable for:[2]
- Hepatoesplenomegaly
- Lymphadenopathy
Laboratory Findings
- Laboratory findings consistent with the diagnosis of aggressive NK-cell leukemia, include:[2]
Peripheral Blood Smear
- Large granular lymphocyte (LGL)
- Fine or coarse azurophilic granules and nucleoli of varying prominence
- Nuclei may be irregular and hyperchromatic
Imaging Findings
- There are no imaging findings associated with aggressive NK-cell leukemia.
Other Diagnostic Studies
- Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy.
- Findings on bone marrow biopsy, include:[2]
- Extensive marrow replacement by leukemic cells
- Reactive histiocytes displaying hemophagocytosis
Treatment
Medical Therapy
- The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.[2]
- L-asparaginase containing chemotherapy regimen followed by allogeneic stem cell transplantation shows to slightly prolong life, but relapse is almost assured.[3]
- Other novel treatments may include pralatrexate.[2]
Surgery
- Surgery is not recommended among patients with aggressive NK-cell leukemia.
Prevention
- There are no primary preventive measures available for aggressive NK-cell leukemia.
References
- ↑ 1.0 1.1 1.2 1.3 [1] Chan JK. "Natural killer cell neoplasms." Anat Pathol. 1998;3:77-145. PMID 10389582
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Aggressive NK-cell leukemia. https://en.wikipedia.org/wiki/Aggressive_NK-cell_leukemia Accessed on May 5, 2016
- ↑ Ishida, F; Ko, YH (2012 June). "Aggressive natural killer cell leukemia: therapeutic potential of L-asparaginase and allogeneic hematopoietic stem cell transplantation". Cancer Sci. 103(6): 1079–83. PMID 22360679. Check date values in:
|date=(help)