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{{SK}} Agranular CD4+CD56+ hematodermic neoplasm; CD4+CD56+ hematodermic neoplasm; HDT; Blastic plasmacytoid dendritic cell neoplasm; BPDCN;  
{{SK}} Agranular CD4+CD56+ hematodermic neoplasm; CD4+CD56+ hematodermic neoplasm; HDT; Blastic plasmacytoid dendritic cell neoplasm; BPDCN;  
==Overview==
==Overview==
Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens. 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==
*Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens.
*Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens.
*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].
==Pathophysiology==
==Pathophysiology==
*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>
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*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The deletion in 5q has been associated with the development of blastic NK cell lymphoma.
*The deletion in 5q has been associated with the development of blastic NK cell lymphoma.
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*On microscopic histopathological analysis, fine chromatin and scanty cytoplasm resembling lymphoblasts, or in some cases, myeloblasts, and may on occasion exhibit sub-membranous cytoplasmic vacuolations surrounding the nucleus are characteristic findings of blastic NK cell lymphoma.
*On microscopic histopathological analysis, fine chromatin and scanty cytoplasm resembling lymphoblasts, or in some cases, myeloblasts, and may on occasion exhibit sub-membranous cytoplasmic vacuolations surrounding the nucleus are characteristic findings of blastic NK cell lymphoma.
*Tumor cells are invariably CD4+ and CD56+, and usually HLA-DR and CD45RA are positive as well. CD2 and CD34 are usually negative; and expression of TdT, CD7 and cytoplasmic CD3 is variable.
*Tumor cells are invariably CD4+ and CD56+, and usually HLA-DR and CD45RA are positive as well. CD2 and CD34 are usually negative; and expression of TdT, CD7 and cytoplasmic CD3 is variable.
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Image:Rt-2014-4-5474-g002 02.jpg|Large malignant-appearing cells, with agranular cytoplasm, cleaved nuclei and prominent neocleoli on peripheral blood smear using Wright stain (A) and similar blast cells present in cerebrospinal fluid
Image:Rt-2014-4-5474-g002 02.jpg|Large malignant-appearing cells, with agranular cytoplasm, cleaved nuclei and prominent neocleoli on peripheral blood smear using Wright stain (A) and similar blast cells present in cerebrospinal fluid
</gallery>
</gallery>
==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==
==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:
*Blastic NK cell lymphoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx2]
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===Gender===
===Gender===
*Males are more commonly affected with blastic NK cell lymphoma than female.
*Males are more commonly affected with blastic NK cell lymphoma than female.
==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==
== Natural History, Complications and Prognosis==
*The majority of patients with Blastic NK cell lymphoma have an aggressive clinical course.
*The majority of patients with Blastic NK cell lymphoma have an aggressive clinical course.
*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 poor, and the patient survives almost 1 year after the diagnosis.
*Prognosis is generally poor, and the patient survives almost 1 year after the diagnosis.
==Diagnosis==
==Diagnosis==
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[[File:Rt-2014-4-5474-g001.jpg|thumb|center|500px|A plaque-like pigmented bruised rash over the patient’s trunk (A) and face (B)<ref name="pmid25568744">{{cite journal| author=Saeed H, Awasthi M, Al-Qaisi A, Massarweh S| title=Blastic plasmacytoid dendritic cell neoplasm with extensive cutaneous and central nervous system involvement. | journal=Rare Tumors | year= 2014 | volume= 6 | issue= 4 | pages= 5474 | pmid=25568744 | doi=10.4081/rt.2014.5474 | pmc=PMC4274438 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25568744  }} </ref>]]
[[File:Rt-2014-4-5474-g001.jpg|thumb|center|500px|A plaque-like pigmented bruised rash over the patient’s trunk (A) and face (B)<ref name="pmid25568744">{{cite journal| author=Saeed H, Awasthi M, Al-Qaisi A, Massarweh S| title=Blastic plasmacytoid dendritic cell neoplasm with extensive cutaneous and central nervous system involvement. | journal=Rare Tumors | year= 2014 | volume= 6 | issue= 4 | pages= 5474 | pmid=25568744 | doi=10.4081/rt.2014.5474 | pmc=PMC4274438 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25568744  }} </ref>]]
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*There are no specific laboratory findings associated with blastic NK cell lymphoma.
*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===
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*There are no imaging study findings associated with blastic NK cell lymphoma
*[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 ===
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Blastic NK cell lymphoma may also be diagnosed using biopsy and immunochemistry.
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*The mainstay of therapy for blastic NK cell lymphoma is chemotherapy with CHOP or COP-like regimens.
*The mainstay of therapy for blastic NK cell lymphoma is chemotherapy with CHOP or COP-like regimens.
*[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 ===
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*There are no primary preventive measures available for blastic NK cell lymphoma.
*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==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 19:56, 10 May 2016

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

Synonyms and keywords: Agranular CD4+CD56+ hematodermic neoplasm; CD4+CD56+ hematodermic neoplasm; HDT; Blastic plasmacytoid dendritic cell neoplasm; BPDCN;

Overview

Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens. Blastic NK cell lymphoma is a type of lymphoma. It does not appear to be associated with Epstein Barr virus.[1]

Historical Perspective

  • Blastic NK cell lymphoma was first discovered by Adachi, an American hematologist, in 1994 following an unusual presentation of cutaneous lymphoma that express CD4 and CD56 antigens but no other T cell and B cell antigens.

Pathophysiology

  • Blastic NK cell lymphoma is a type of lymphoma. It does not appear to be associated with Epstein Barr virus.[1]
  • Blastic NK cell lymphoma is currently classified by World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues as an aggressive neoplasm derived from the precursors of plasmacytoid dendritic cells and categorically placed under the heading “Acute myeloid leukemia (AML) and related precursor neoplasms.”
  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The deletion in 5q has been associated with the development of blastic NK cell lymphoma.
  • On microscopic histopathological analysis, fine chromatin and scanty cytoplasm resembling lymphoblasts, or in some cases, myeloblasts, and may on occasion exhibit sub-membranous cytoplasmic vacuolations surrounding the nucleus are characteristic findings of blastic NK cell lymphoma.
  • Tumor cells are invariably CD4+ and CD56+, and usually HLA-DR and CD45RA are positive as well. CD2 and CD34 are usually negative; and expression of TdT, CD7 and cytoplasmic CD3 is variable.

Differentiating [disease name] from other Diseases

  • Blastic NK cell lymphoma 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 blastic NK cell lymphoma is unknown as it is an extremely rare disorder.[2]

Age

  • Blastic NK cell lymphoma is more commonly observed amongmiddle-aged or elderly patients. The mean age at diagnosis is 66 years.[3]

Gender

  • Males are more commonly affected with blastic NK cell lymphoma than female.

Natural History, Complications and Prognosis

  • The majority of patients with Blastic NK cell lymphoma have an aggressive clinical course.
  • Prognosis is generally poor, and the patient survives almost 1 year after the diagnosis.

Diagnosis

Cells are positive for CD4 and CD56.[4][5]

Symptoms

  • Symptoms of blastic NK cell lymphoma may include the following:
  • Nodules, plaques and patches of variable sizes on skin

Physical Examination

  • Physical examination may be remarkable for:
  • Nodules, plaques and patches of variable sizes on skin
A plaque-like pigmented bruised rash over the patient’s trunk (A) and face (B)[2]

Laboratory Findings

  • There are no specific laboratory findings associated with blastic NK cell lymphoma.

Imaging Findings

  • There are no imaging study findings associated with blastic NK cell lymphoma

Other Diagnostic Studies

  • Blastic NK cell lymphoma may also be diagnosed using biopsy and immunochemistry.

Treatment

Medical Therapy

  • The mainstay of therapy for blastic NK cell lymphoma is chemotherapy with CHOP or COP-like regimens.

Prevention

  • There are no primary preventive measures available for blastic NK cell lymphoma.

References

  1. 1.0 1.1 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. 2.0 2.1 Saeed H, Awasthi M, Al-Qaisi A, Massarweh S (2014). "Blastic plasmacytoid dendritic cell neoplasm with extensive cutaneous and central nervous system involvement". Rare Tumors. 6 (4): 5474. doi:10.4081/rt.2014.5474. PMC 4274438. PMID 25568744.
  3. Hou, Steve; Jaworski, Joseph; Swami, Vanlila; Heintzelman, Rebecca; Cusack, Carrie; Chung, Christina; Peck, Jeremy; Fanelli, Matthew; Styler, Michael; Rizk, Sanaa (2015). "Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation". Pathology and Laboratory Medicine International: 7. doi:10.2147/PLMI.S71492. ISSN 1179-2698.
  4. 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)
  5. 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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