Anaplastic large cell lymphoma, ALK positive: Difference between revisions

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__NOTOC__
#REDIRECT[[Anaplastic large cell lymphoma]]
{{SI}}
{{DiseaseDisorder infobox |
  Name          = auses
 
Anaplastic large cell lymphoma, ALK positive
 
ls|
  ICD10          = {{ICD10|C|84|4|c|81}} |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
}}
{{CMG}}; {{AE}} {{AP}}
 
{{SK}} ALCL-ALK(+)
 
==Overview==
The Anaplastic large cell lymphoma ALK-positive ('''A'''naplastic '''L'''lymphoma '''K'''inase) consist of [[CD30]]-positive T-cells with abundant cytoplasm, a pleomorphic nucleus (horseshoe-shaped nucleus) and a eosinophilic paranuclear region<ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693  }} </ref>. This ALK-positive lymphoma has a translocation in the ALK gene [T(2;5)(p23;q35)], which will in turn, express the ALK protein<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>.
 
==Historical Perspective==
 
==Classification==
===Morphologic Classification<ref>{{cite web|url=http://go.galegroup.com/ps/retrieve.do?sgHitCountType=None&sort=RELEVANCE&inPS=true&prodId=HRCA&userGroupName=mlin_b_bethidmc&tabID=T002&searchId=R1&resultListType=RESULT_LIST&contentSegment=&searchType=AdvancedSearchForm&currentPosition=1&contentSet=GALE%7CA188154738&&docId=GALE|A188154738&docType=GALE&role=|title=The anaplastic lymphoma kinase in the pathogenesis of cancer}}</ref>===
====Classical Variants====
*'''Common pattern''' ALK positive anaplastic large cell lymphoma
 
====Atypical Variants====
*'''Small cell''' ALK positive anaplastic large cell lymphoma
*'''Lymphohistiocytic''' ALK positive anaplastic large cell lymphoma
*'''Giant cell''' ALK positive anaplastic large cell lymphoma
*'''Hodgkin's like''' ALK positive anaplastic large cell lymphoma
 
====Rare Variants====
*'''Sarcomatoid''' ALK positive anaplastic large cell lymphoma
 
==Pathophysiology ==
ALCL morphologic features are variable, which allowed to classify this entity into five morphologic patterns<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>:
*'''"Common" pattern''': Its the most common morphologic variant (75%)<ref name="pmid9736036">{{cite journal| author=Falini B, Bigerna B, Fizzotti M, Pulford K, Pileri SA, Delsol G et al.| title=ALK expression defines a distinct group of T/null lymphomas ("ALK lymphomas") with a wide morphological spectrum. | journal=Am J Pathol | year= 1998 | volume= 153 | issue= 3 | pages= 875-86 | pmid=9736036 | doi=10.1016/S0002-9440(10)65629-5 | pmc=PMC1853018 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9736036  }}</ref>. Cytoplasm may be basophilic or eosinophilic and the cell might have many nuclei with dispersed or clumped chromatin. In large cells, nucleoli tends to be more prominent. Given that the lymphomatous cells grow in the lymph node's sinuses, it may resemble a metastatic tumor.
*'''Lymphohistiocytic pattern (10%)''': histiocytes with acidophilic cytoplasm and a perinuclear clear area, which represents the [[Golgi apparatus]]. This cells have eccentric nuclei with condensed chromatin<ref>{{cite web|url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1858171/pdf/amjpathol00028-0072.pdf|title=
Frequent Expression ofthe NPM-ALK Chimeric Fusion Protein inAnaplastic Large-Cell Lymphoma, Lympho-Histiocytic Type}}</ref>.  Lymphomatous cells tend tu cluster around the perivascular area, evidenced by immunostaining with CD30 and ALK antibodies<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>.
*'''Hodgkin's like pattern (3.3%)''': Morphological characteristics reassemble [[Hodgkin's lymphoma]], variant nodular sclerosis<ref name="pmid16434897">{{cite journal| author=Vassallo J, Lamant L, Brugieres L, Gaillard F, Campo E, Brousset P et al.| title=ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin's lymphoma: report of 10 cases. | journal=Am J Surg Pathol | year= 2006 | volume= 30 | issue= 2 | pages= 223-9 | pmid=16434897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16434897  }} </ref>. Predominates in female patients over male patients.
**Immunophenotype<ref name="pmid16434897">{{cite journal| author=Vassallo J, Lamant L, Brugieres L, Gaillard F, Campo E, Brousset P et al.| title=ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin's lymphoma: report of 10 cases. | journal=Am J Surg Pathol | year= 2006 | volume= 30 | issue= 2 | pages= 223-9 | pmid=16434897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16434897  }} </ref>
***Positive: [[CD30]], [[ALK]], [[epithelial membrane antigen]] ([[EMA]]), [[CD43]] (only 66% of the times) and [[perforin]].
***Negative: [[CD15]], [[CD20]], Pax5/BSAP and [[EBV]].
*'''Small cell pattern (8.3%)''': Present nuclear irregularity and perivascular/intravascular distribution<ref name="pmid8394652">{{cite journal| author=Kinney MC, Collins RD, Greer JP, Whitlock JA, Sioutos N, Kadin ME| title=A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma. | journal=Am J Surg Pathol | year= 1993 | volume= 17 | issue= 9 | pages= 859-68 | pmid=8394652 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8394652  }} </ref>. Occasionally, this lymphomatous cells have pale cytoplasm with a central nucleus ("fried egg cell")<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>.
*'''Giant cell pattern (3.3%)'''
 
<i>One single patient may present more than one morphologic pattern in the same or progressive biopsies</i><ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693  }} </ref>.
 
==Causes==
 
The ALK positive Anaplastic large cell lymphoma have a rearrangement in the Anaplastic Lymphoma Kinase (ALK) gene. The most frequent gene translocation is T(2;5)(p23;q35)<ref name="pmid8122112">{{cite journal| author=Morris SW, Kirstein MN, Valentine MB, Dittmer KG, Shapiro DN, Saltman DL et al.| title=Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin's lymphoma. | journal=Science | year= 1994 | volume= 263 | issue= 5151 | pages= 1281-4 | pmid=8122112 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8122112  }} </ref>. This translocation produces a chimeric protein between the nucleolar phosphoprotein gene (5q35) a ALK gene (2p23). The new protein has structural similarity to the [[insulin growth factor receptor]].<ref>{{cite web|url=http://go.galegroup.com/ps/retrieve.do?sgHitCountType=None&sort=RELEVANCE&inPS=true&prodId=HRCA&userGroupName=mlin_b_bethidmc&tabID=T002&searchId=R2&resultListType=RESULT_LIST&contentSegment=&searchType=AdvancedSearchForm&currentPosition=1&contentSet=GALE%7CA15341631&&docId=GALE|A15341631&docType=GALE&role=|title=Fusion of kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin's lymphoma}}</ref>
 
== Differential Diagnosis ==
 
== Epidemiology and Demographics==
This entity affects primarily young, male patients<ref name="pmid11090048">{{cite journal| author=Stein H, Foss HD, Dürkop H, Marafioti T, Delsol G, Pulford K et al.| title=CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features. | journal=Blood | year= 2000 | volume= 96 | issue= 12 | pages= 3681-95 | pmid=11090048 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11090048  }} </ref> and accounts for 3% of all [[NHL]], 40% of all large cell lymphomas<ref>{{cite web|url=http://www.bloodjournal.org/content/93/8/2697?sso-checked=true|title=ALK+ Lymphoma: Clinico-Pathological Findings and Outcome}}</ref> and 10%-20% of childhood lymphomas, although it is particularly incident in patients between 10 and 29 years<ref>{{cite web|url=http://www.bloodjournal.org/content/93/8/2697?sso-checked=true|title=ALK+ Lymphoma: Clinico-Pathological Findings and Outcome}}</ref>.
 
A more recent study evaluated 1,320 cases of [[peripheral T-cell lymphomas]] and [[NK cell lymphomas]] between 1990 and 2002 in 22 different centers, concluding that the ALK-Positive ALCL is the fifth most incident (6.6% of total patients)<ref>{{cite web|url=http://jco.ascopubs.org/content/26/25/4124.full.pdf|title=International Peripheral T-Cell and Natural Killer/T-Cell Lymphoma Study: Pathology Findings and Clinical Outcomes}}</ref>, although in the United states, it's the most frequent type of [[peripheral T-cell lymphoma]].
 
== Risk Factors==
 
== Natural History, Complications and Prognosis==
 
== Diagnosis ==
 
===History and Symptoms===
Most adult patients present with painless [[lymphadenopathy]]. Although retroperitoneal and peripheral<ref name="pmid25674293">{{cite journal| author=Yu G, Gao Z, Huang X| title=ALK-positive anaplastic large cell lymphoma with an unusual alveolar growth pattern. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 12 | pages= 9086-9 | pmid=25674293 | doi= | pmc=PMC4314028 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25674293  }} </ref> [[lymphadenopathy]] is very common, other possible locations include the  gastrointestinal tract, breast<ref name="pmid25490539">{{cite journal| author=Gidengil CA, Predmore Z, Mattke S, van Busum K, Kim B| title=Breast implant-associated anaplastic large cell lymphoma: a systematic review. | journal=Plast Reconstr Surg | year= 2014 | volume=  | issue=  | pages=  | pmid=25490539 | doi=10.1097/PRS.0000000000001037 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25490539  }} </ref>, spleen<ref name="pmid11097375">{{cite journal| author=Hebeda KM, MacKenzie MA, van Krieken JH| title=A case of anaplastic lymphoma kinase-positive anaplastic large cell lymphoma presenting with spontaneous splenic rupture: an extremely unusual presentation. | journal=Virchows Arch | year= 2000 | volume= 437 | issue= 4 | pages= 459-64 | pmid=11097375 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11097375  }} </ref>, liver<ref name="pmid17396261">{{cite journal| author=Grewal JS, Smith LB, Winegarden JD, Krauss JC, Tworek JA, Schnitzer B| title=Highly aggressive ALK-positive anaplastic large cell lymphoma with a leukemic phase and multi-organ involvement: a report of three cases and a review of the literature. | journal=Ann Hematol | year= 2007 | volume= 86 | issue= 7 | pages= 499-508 | pmid=17396261 | doi=10.1007/s00277-007-0289-3 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17396261  }} </ref>, bone<ref name="pmid17396261">{{cite journal| author=Grewal JS, Smith LB, Winegarden JD, Krauss JC, Tworek JA, Schnitzer B| title=Highly aggressive ALK-positive anaplastic large cell lymphoma with a leukemic phase and multi-organ involvement: a report of three cases and a review of the literature. | journal=Ann Hematol | year= 2007 | volume= 86 | issue= 7 | pages= 499-508 | pmid=17396261 | doi=10.1007/s00277-007-0289-3 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17396261  }} </ref> and respiratory tract<ref name="pmid18670314">{{cite journal| author=Tan DS, Eng PC, Lim ST, Thye LS, Tao M| title=Primary tracheal lymphoma causing respiratory failure. | journal=J Thorac Oncol | year= 2008 | volume= 3 | issue= 8 | pages= 929-30 | pmid=18670314 | doi=10.1097/JTO.0b013e318180271d | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18670314  }} </ref>.
 
Common symptoms include typical [[B symptoms]]<ref>{{cite web|url=http://www.lymphomas.org.uk/sites/default/files/pdfs/T-cell-lymphomas.pdf|title=T-cell lymphomas (Lymphoma Association)}}</ref>([[Fever]], [[weight loss]] and [[night sweats]]).
 
===Laboratory Findings<ref>{{cite web|url=http://www.bloodjournal.org/content/111/12/5496|title=ALK− anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}}</ref>===
*[[Anemia]]
*[[Thrombocytopenia]]
*Elevated [[lactate dehydrogenase]] ([[LDH]]) levels
 
== Treatment ==
 
==References==
{{Reflist|2}}

Latest revision as of 14:19, 8 October 2015

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