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| __NOTOC__
| | #REDIRECT[[Anaplastic large cell lymphoma]] |
| {{SI}}
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| {{DiseaseDisorder infobox |
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| Name = auses
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| Anaplastic large cell lymphoma, ALK positive
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| ls|
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| ICD10 = {{ICD10|C|84|4|c|81}} |
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| OMIM = |
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| MedlinePlus = |
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| eMedicineSubj = |
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| {{CMG}}; {{AE}} {{AP}}
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| {{SK}} ALCL-ALK(+)
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| ==Overview==
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| 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>.
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| ==Historical Perspective==
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| ==Classification==
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| ==Pathophysiology ==
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| 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>:
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| *'''"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.
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| *'''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=
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| 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>.
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| *'''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>.
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| *'''Giant cell pattern (3.3%)''':
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| *'''Hodgkin's like pattern (3.3%)''':
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| 1033
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| 1152
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| ==Causes==
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| == Differential Diagnosis ==
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| == Epidemiology and Demographics==
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| 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>.
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| == Risk Factors==
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| == Natural History, Complications and Prognosis==
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| == Diagnosis ==
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| ===History and Symptoms===
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| == Treatment ==
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| ==References==
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| {{Reflist|2}}
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