Anaplastic Large Cell Lymphoma (ALK+/ALK−): Difference between revisions
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==Primary Author(s)*== | ==Primary Author(s)*== | ||
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==WHO Classification of Disease== | ==WHO Classification of Disease<ref name=":0">Li, W. The 5th Edition of the World Health Organization Classification of Hematolymphoid Tumors. In Leukemia; Weijie, L., Ed.; Exon Publications: Brisbane, Australia, 2022; pp. 1–21, <nowiki>ISBN 978-0-645-33207-0</nowiki>.</ref>== | ||
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==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
Anaplastic large cell lymphoma (ALCL) is a distinctive CD30-positive peripheral T-cell lymphoma that is rare in the CNS and is separated into two distinct types: ALK-positive (ALK+ ALCL) and ALK-negative (ALK− ALCL). | Anaplastic large cell lymphoma (ALCL) is a distinctive CD30-positive peripheral T-cell lymphoma that is rare in the CNS and is separated into two distinct types: ALK-positive (ALK+ ALCL) and ALK-negative (ALK− ALCL).<ref name=":0" /> | ||
==Synonyms / Terminology== | ==Synonyms / Terminology== | ||
None | None | ||
==Epidemiology / Prevalence== | ==Epidemiology / Prevalence== | ||
ALK+ ALCL occurs from early childhood to young adulthood with a male preponderance. | ALK+ ALCL occurs from early childhood to young adulthood with a male preponderance.<ref name=":1">George, D.H.; Scheithauer, B.W.; Aker, F.V.; Kurtin, P.J.; Burger, P.C.; Cameselle-Teijeiro, J.; McLendon, R.E.; Parisi, J.E.; Paulus, W.; Roggendorf, W.; et al. Primary Anaplastic Large Cell Lymphoma of the Central Nervous System: Prognostic Effect of ALK-1 Expression. Am. J. Surg. Pathol. 2003, 27, 487–493</ref> | ||
ALK− ALCL affects adults (median age: 65 years), also with a male preponderance. | ALK− ALCL affects adults (median age: 65 years), also with a male preponderance. | ||
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==Sites of Involvement== | ==Sites of Involvement== | ||
ALK+ ALCL occurs as single or multiple supratentorial parenchymal lesions with or without infratentorial involvement, and rarely with spinal cord involvement. Extension to involve the meninges and (rarely) the skull can occur. | ALK+ ALCL occurs as single or multiple supratentorial parenchymal lesions with or without infratentorial involvement, and rarely with spinal cord involvement. Extension to involve the meninges and (rarely) the skull can occur.<ref>Karikari, I.O.; Thomas, K.K.; Lagoo, A.; Cummings, T.J.; George, T.M. Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma in a Child. Pediatr. Neurosurg. 2007, 43, 516–521. [CrossRef] [PubMed]</ref> | ||
ALK− ALCL occurs as single or multiple lesions, usually supratentorial | ALK− ALCL occurs as single or multiple lesions, usually supratentorial | ||
==Morphologic Features== | ==Morphologic Features== | ||
ALK+ ALCL shows a diffuse proliferation of large atypical cells with abundant cytoplasm, including hallmark cells with bean-shaped nuclei and an eosinophilic paranuclear area | ALK+ ALCL shows a diffuse proliferation of large atypical cells with abundant cytoplasm, including hallmark cells with bean-shaped nuclei and an eosinophilic paranuclear area<ref>Jaffe ES. Anaplastic large cell lymphoma: the shifting sands of diagnostic hematopathology. Modern Pathol. 2001;14:219-228.</ref> | ||
The cerebrospinal fluid may be involved. The large atypical neoplastic cells may have cytoplasmic azurophilic granules | The cerebrospinal fluid may be involved. The large atypical neoplastic cells may have cytoplasmic azurophilic granules | ||
==Immunophenotype== | ==Immunophenotype== | ||
Put your text here and fill in the table | Put your text here and fill in the table<ref>Kadin ME. Primary Ki-1-positive anaplastic large-cell lymphoma: a distinct clinicopathologic entity. Ann Oncol. 1994;5:S25-S30.</ref> | ||
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|t(2;5)(p23;q35)||''NPM1''::''ALK'' fusion | |t(2;5)(p23;q35)||''NPM1''::''ALK'' fusion | ||
|5' NPM1::3' ALK on der(5). constitutive activation of the catalytic domain of ALK. Kinase function activated by oligomerization of NPM1::ALK mediated by the NPM1 portion|| | |5' NPM1::3' ALK on der(5). constitutive activation of the catalytic domain of ALK. Kinase function activated by oligomerization of NPM1::ALK mediated by the NPM1 portion<ref>Geetha, N.; Sreelesh, K.P.; Nair, R.; Mathews, A. Anaplastic large cell lymphoma presenting as a cerebellar mass. Hematol. Oncol. Stem Cell Ther. 2014, 7, 157–161. </ref><ref>Benharroch D, Meguerian-Bedoyan Z, Lamant L, et al. ALK-positive lymphoma: a single disease with a broad spectrum of morphology. Blood. 1998;91:2076-2084.</ref>||30 to 50% of ALCL<ref name=":0" /> | ||
( | (COSF198) | ||
( | (<ref>John G Tate, Sally Bamford, Harry C Jubb, Zbyslaw Sondka, David M Beare, Nidhi Bindal, Harry Boutselakis, Charlotte G Cole, Celestino Creatore, Elisabeth Dawson, Peter Fish, Bhavana Harsha, Charlie Hathaway, Steve C Jupe, Chai Yin Kok, Kate Noble, Laura Ponting, Christopher C Ramshaw, Claire E Rye, Helen E Speedy, Ray Stefancsik, Sam L Thompson, Shicai Wang, Sari Ward, Peter J Campbell, Simon A Forbes, COSMIC: the Catalogue Of Somatic Mutations In Cancer, ''Nucleic Acids Research'', Volume 47, Issue D1, 08 January 2019, Pages D941–D947,</ref>) | ||
|Yes | |Yes | ||
|Yes | |Yes | ||
|Yes | |Yes | ||
| | |Localized in both cytoplasm and nucleus.<ref name=":0" /> | ||
Localized in both cytoplasm and nucleus. | |||
In translocations other than the t(2;5), i.e. in t(2;Var) involving various partners and ALK, the fusion protein has a cytoplasmic localization; they are therefore called "cytoplasm only" ALK+ ALCL. | In translocations other than the t(2;5), i.e. in t(2;Var) involving various partners and ALK, the fusion protein has a cytoplasmic localization; they are therefore called "cytoplasm only" ALK+ ALCL.<ref name=":0" /> | ||
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|t(X;2)(q11;p23) | |t(X;2)(q11;p23) | ||
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|For the t(X;2) translocation, localization is restricted to the membrane. | |For the t(X;2) translocation, localization is restricted to the membrane.<ref name=":0" /> | ||
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|t(1;2)(q25;p23) | |t(1;2)(q25;p23) | ||
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|TPM3::ALK is constitutively activated | |TPM3::ALK is constitutively activated<ref name=":0" /> | ||
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|inv(2)(p23q35) | |inv(2)(p23q35) | ||
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|ider(2)(q10)inv(2) has been found in some cases, carrying 2 additional copies of the ATIC::ALK hybrid gene; frequent complex karyotypes | |ider(2)(q10)inv(2) has been found in some cases, carrying 2 additional copies of the ATIC::ALK hybrid gene; frequent complex karyotypes<ref name=":0" /> | ||
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|t(2;3)(p23;q21) | |t(2;3)(p23;q21) | ||
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|the localization is restricted to granules (vesicles) in the cytoplasm | |the localization is restricted to granules (vesicles) in the cytoplasm<ref name=":0" /> | ||
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==Individual Region Genomic Gain/Loss/LOH== | ==Individual Region Genomic Gain/Loss/LOH== | ||
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==References== | ==References== | ||
<references />(use "Cite" icon at top of page) | <references />(use "Cite" icon at top of page) | ||
===EXAMPLE Book=== | ===<ref name=":1" />EXAMPLE Book=== | ||
#Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171. | #Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171. | ||