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==
 
==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 cell, nucleoli tends to be more prominent.
 
*Lymphohistiocytic patter (10%)
*Small cell (8.3%)
*Giant cell (3.3%)
*Hodgkin's like pattern (3.3%)
 
==Causes==
 
== 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>.
 
== Risk Factors==
 
== Natural History, Complications and Prognosis==
 
== Diagnosis ==
 
===History and Symptoms===
 
== Treatment ==
 
==References==
{{Reflist|2}}

Latest revision as of 14:19, 8 October 2015