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Blastic NK cell lymphoma is a type of [[lymphoma]]. It does not appear to be associated with [[Epstein Barr virus]].<ref name="pmid9192774">{{cite journal |author=Chan JK, Sin VC, Wong KF, ''et al'' |title=Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm |journal=Blood |volume=89 |issue=12 |pages=4501–13 |year=1997 |month=June |pmid=9192774 |doi= |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=9192774}}</ref>
Blastic NK cell lymphoma is a type of [[lymphoma]]. It does not appear to be associated with [[Epstein Barr virus]].<ref name="pmid9192774">{{cite journal |author=Chan JK, Sin VC, Wong KF, ''et al'' |title=Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm |journal=Blood |volume=89 |issue=12 |pages=4501–13 |year=1997 |month=June |pmid=9192774 |doi= |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=9192774}}</ref>
==Historical Perspective==
==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
[nationality + occupation], in [year] during/following [event].
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], [gene] mutations were first identified in the pathogenesis
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
of [disease name].
*In [year], the first [discovery] was developed by [scientist] to
treat/diagnose [disease name].
==Classification==
==Classification==
*[Disease name] may be classified according to [classification method]
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
into [number] subtypes/groups:
:*[group1]
:*[group1]
:*[group2]
:*[group2]
:*[group3]
:*[group3]
*Other variants of [disease name] include [disease subtype 1],
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
[disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [disease name] is characterized by [feature1],
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
[feature2], and [feature3].
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*The [gene name] gene/Mutation in [gene name] has been associated with
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
the development of [disease name], involving the [molecular pathway]
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
pathway.
*On gross pathology, [feature1], [feature2], and [feature3] are
characteristic findings of [disease name].
*On microscopic histopathological analysis, [feature1], [feature2],
and [feature3] are characteristic findings of [disease name].
==Causes==
==Causes==
* [Disease name] may be caused by either [cause1], [cause2], or
* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
[cause3].
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* [Disease name] is caused by a mutation in the [gene1], [gene2], or
[gene3] gene[s].
* There are no established causes for [disease name].
* There are no established causes for [disease name].
==Differentiating [disease name] from other Diseases==
==Differentiating [disease name] from other Diseases==
*[Disease name] must be differentiated from other diseases that cause
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
[clinical feature 1], [clinical feature 2], and [clinical feature 3],
such as:
:*[Differential dx1]
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx2]
:*[Differential dx3]
:*[Differential dx3]
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range]
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
per 100,000 individuals worldwide.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
* In [year], the incidence of [disease name] was estimated to be
[number or range] cases per 100,000 individuals in [location].
===Age===
===Age===
*Patients of all age groups may develop [disease name].
*Patients of all age groups may develop [disease name].
*[Disease name] is more commonly observed among patients aged [age
*[Disease name] is more commonly observed among patients aged [age range] years old.
range] years old.
*[Disease name] is more commonly observed among [elderly patients/young patients/children].
*[Disease name] is more commonly observed among [elderly
patients/young patients/children].
===Gender===
===Gender===
*[Disease name] affects men and women equally.
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
[gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to
1.
===Race===
===Race===
*There is no racial predilection for [disease name].
*There is no racial predilection for [disease name].
Line 67: Line 47:
*[Race 2] individuals are less likely to develop [disease name].
*[Race 2] individuals are less likely to develop [disease name].
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for
*The majority of patients with [disease name] remain asymptomatic for [duration/years].
[duration/years].
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*Early clinical features include [manifestation 1], [manifestation 2],
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*If left untreated, [#%] of patients with [disease name] may progress
*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
to develop [manifestation 1], [manifestation 2], and [manifestation
3].
*Common complications of [disease name] include [complication 1],
[complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10year
mortality/survival rate] of patients with [disease name] is
approximately [#%].
== Diagnosis ==
== Diagnosis ==
==Diagnosis==
==Diagnosis==
Cells are positive for [[CD4]] and [[CD56]].<ref name="pmid16434387">{{cite journal |author=Ng AP, Lade S, Rutherford T, McCormack C, Prince HM, Westerman DA |title=Primary cutaneous CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma): a report of five cases |journal=Haematologica |volume=91 |issue=1 |pages=143–4 |year=2006 |month=January |pmid=16434387 |doi= |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=16434387}}</ref><ref name="pmid15832009">{{cite journal |author=Kim Y, Kang MS, Kim CW, Sung R, Ko YH |title=CD4+CD56+ lineage negative hematopoietic neoplasm: so called blastic NK cell lymphoma |journal=J. Korean Med. Sci. |volume=20 |issue=2 |pages=319–24 |year=2005 |month=April |pmid=15832009 |doi= |url=http://jkms.org/contents/jkms.php?pubyear=2005&vol=20&fpage=319}}</ref>
Cells are positive for [[CD4]] and [[CD56]].<ref name="pmid16434387">{{cite journal |author=Ng AP, Lade S, Rutherford T, McCormack C, Prince HM, Westerman DA |title=Primary cutaneous CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma): a report of five cases |journal=Haematologica |volume=91 |issue=1 |pages=143–4 |year=2006 |month=January |pmid=16434387 |doi= |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=16434387}}</ref><ref name="pmid15832009">{{cite journal |author=Kim Y, Kang MS, Kim CW, Sung R, Ko YH |title=CD4+CD56+ lineage negative hematopoietic neoplasm: so called blastic NK cell lymphoma |journal=J. Korean Med. Sci. |volume=20 |issue=2 |pages=319–24 |year=2005 |month=April |pmid=15832009 |doi= |url=http://jkms.org/contents/jkms.php?pubyear=2005&vol=20&fpage=319}}</ref>
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 1]
:*[criterion 2]
:*[criterion 2]
Line 111: Line 82:
:*[finding 6]
:*[finding 6]
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease
*There are no specific laboratory findings associated with [disease name].
name].
*A [positive/negative] [test name] is diagnostic of [disease name].
*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
[serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
name].
*Other laboratory findings consistent with the diagnosis of [disease
name] include [abnormal test 1], [abnormal test 2], and [abnormal test
3].
===Imaging Findings===
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*There are no [imaging study] findings associated with [disease name].
*[Imaging study 1] is the imaging modality of choice for [disease
*[Imaging study 1] is the imaging modality of choice for [disease name].
name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*On [imaging study 1], [disease name] is characterized by [finding 1],
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
[finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and
[finding 3].
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2],
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
and [finding 3].
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
supportive care.
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*The mainstay of therapy for [disease name] is [medical therapy 1] and
[medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
finding/imaging] every [frequency/duration].
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
*[Surgical procedure] can only be performed for patients with [disease
stage] [disease name].
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for [disease
*There are no primary preventive measures available for [disease name].
name].
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
*Effective measures for the primary prevention of [disease name]
*Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].
include [measure1], [measure2], and [measure3].
*Once diagnosed and successfully treated, patients with [disease name]
are followedup
every [duration]. Followup
testing includes [test 1],
[test 2], and [test 3].
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 13:37, 10 May 2016

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List of terms related to Blastic NK cell lymphoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Blastic NK cell lymphoma is a type of lymphoma. It does not appear to be associated with Epstein Barr virus.[1]

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

  • [Disease name] may be caused by either [cause1], [cause2], or [cause3].
  • [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnosis

Cells are positive for CD4 and CD56.[2][3]

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].

References

  1. Chan JK, Sin VC, Wong KF; et al. (1997). "Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm". Blood. 89 (12): 4501–13. PMID 9192774. Unknown parameter |month= ignored (help)
  2. Ng AP, Lade S, Rutherford T, McCormack C, Prince HM, Westerman DA (2006). "Primary cutaneous CD4+/CD56+ hematodermic neoplasm (blastic NK-cell lymphoma): a report of five cases". Haematologica. 91 (1): 143–4. PMID 16434387. Unknown parameter |month= ignored (help)
  3. Kim Y, Kang MS, Kim CW, Sung R, Ko YH (2005). "CD4+CD56+ lineage negative hematopoietic neoplasm: so called blastic NK cell lymphoma". J. Korean Med. Sci. 20 (2): 319–24. PMID 15832009. Unknown parameter |month= ignored (help)

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