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Primary Systemic Anaplastic Large Cell Lymphoma, ALK-Negative

MedGen UID:
235496
•Concept ID:
C1335491
•
Neoplastic Process
Synonym: Primary Systemic ALK-Negative Anaplastic Large Cell Lymphoma

Definition

A CD30 (Ki-1)-positive anaplastic large cell lymphoma with cytoplasmic and/or nuclear expression of the ALK (anaplastic lymphoma kinase) protein. It usually affects both the lymph nodes and extranodal sites (primarily skin, bones, lungs, and soft tissues), and has been associated with a favorable clinical outcome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Systemic Anaplastic Large Cell Lymphoma, ALK-Negative

Professional guidelines

PubMed

Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Clinical Practice Recommendations on Indication and Timing of Hematopoietic Cell Transplantation in Mature T Cell and NK/T Cell Lymphomas: An International Collaborative Effort on Behalf of the Guidelines Committee of the American Society for Blood and Marrow Transplantation.
Kharfan-Dabaja MA, Kumar A, Ayala E, Hamadani M, Reimer P, Gisselbrecht C, d'Amore F, Jantunen E, Ishida T, Bazarbachi A, Foss F, Advani R, Fenske TS, Lazarus HM, Friedberg JW, Aljurf M, Sokol L, Tobinai K, Tse E, Burns LJ, Chavez JC, Reddy NM, Suzuki R, Ahmed S, Nademanee A, Mohty M, Gopal AK, Fanale MA, Pro B, Moskowitz AJ, Sureda A, Perales MA, Carpenter PA, Savani BN
Biol Blood Marrow Transplant 2017 Nov;23(11):1826-1838. Epub 2017 Aug 7 doi: 10.1016/j.bbmt.2017.07.027. PMID: 28797780
ALK-negative systemic anaplastic large cell lymphoma: differential diagnostic and prognostic aspects--a review.
ten Berge RL, Oudejans JJ, Ossenkoppele GJ, Meijer CJ
J Pathol 2003 May;200(1):4-15. doi: 10.1002/path.1331. PMID: 12692835

Recent clinical studies

Etiology

Pathobiology of nodal peripheral T-cell lymphomas: current understanding and future directions.
Bisig B, Savage KJ, De Leval L
Haematologica 2023 Dec 1;108(12):3227-3243. doi: 10.3324/haematol.2023.282716. PMID: 38037800Free PMC Article
Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas.
Feldman AL, Laurent C, Narbaitz M, Nakamura S, Chan WC, de Leval L, Gaulard P
Virchows Arch 2023 Jan;482(1):265-279. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03412-6. PMID: 36210383
A suggested immunohistochemical algorithm for the classification of T-cell lymphomas involving lymph nodes.
Vega F, Medeiros LJ
Hum Pathol 2020 Aug;102:104-116. Epub 2020 May 29 doi: 10.1016/j.humpath.2020.05.006. PMID: 32479842
Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous.
Xing X, Feldman AL
Adv Anat Pathol 2015 Jan;22(1):29-49. doi: 10.1097/PAP.0000000000000047. PMID: 25461779

Diagnosis

Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas.
Feldman AL, Laurent C, Narbaitz M, Nakamura S, Chan WC, de Leval L, Gaulard P
Virchows Arch 2023 Jan;482(1):265-279. Epub 2022 Oct 10 doi: 10.1007/s00428-022-03412-6. PMID: 36210383
ALK-negative CNS anaplastic large cell lymphoma: case report and review of literature.
Lannon M, Lu JQ, Chum M, Wang BH
Br J Neurosurg 2023 Oct;37(5):1245-1250. Epub 2020 Nov 30 doi: 10.1080/02688697.2020.1839630. PMID: 33253051
A suggested immunohistochemical algorithm for the classification of T-cell lymphomas involving lymph nodes.
Vega F, Medeiros LJ
Hum Pathol 2020 Aug;102:104-116. Epub 2020 May 29 doi: 10.1016/j.humpath.2020.05.006. PMID: 32479842
Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
ALK-negative systemic anaplastic large cell lymphoma: differential diagnostic and prognostic aspects--a review.
ten Berge RL, Oudejans JJ, Ossenkoppele GJ, Meijer CJ
J Pathol 2003 May;200(1):4-15. doi: 10.1002/path.1331. PMID: 12692835

Therapy

Non-Hodgkin Lymphoma Developed Shortly after mRNA COVID-19 Vaccination: Report of a Case and Review of the Literature.
Cavanna L, Grassi SO, Ruffini L, Michieletti E, Carella E, Palli D, Zangrandi A, Inzerilli N, Bernuzzi P, Di Nunzio C, Citterio C
Medicina (Kaunas) 2023 Jan 12;59(1) doi: 10.3390/medicina59010157. PMID: 36676781Free PMC Article
Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Lymphomas Associated with Breast Implants: A Review of the Literature.
Rupani A, Frame JD, Kamel D
Aesthet Surg J 2015 Jul;35(5):533-44. doi: 10.1093/asj/sjv016. PMID: 26116741
Anaplastic large cell lymphoma and breast implants: a systematic review.
Kim B, Roth C, Chung KC, Young VL, van Busum K, Schnyer C, Mattke S
Plast Reconstr Surg 2011 Jun;127(6):2141-2150. doi: 10.1097/PRS.0b013e3182172418. PMID: 21358562
Primary cutaneous and systemic anaplastic large cell lymphoma: clinicopathologic aspects and therapeutic options.
Querfeld C, Khan I, Mahon B, Nelson BP, Rosen ST, Evens AM
Oncology (Williston Park) 2010 Jun;24(7):574-87. PMID: 20669794

Prognosis

Pathobiology of nodal peripheral T-cell lymphomas: current understanding and future directions.
Bisig B, Savage KJ, De Leval L
Haematologica 2023 Dec 1;108(12):3227-3243. doi: 10.3324/haematol.2023.282716. PMID: 38037800Free PMC Article
Super-enhancer-based identification of a BATF3/IL-2R-module reveals vulnerabilities in anaplastic large cell lymphoma.
Liang HC, Costanza M, Prutsch N, Zimmerman MW, Gurnhofer E, Montes-Mojarro IA, Abraham BJ, Prokoph N, Stoiber S, Tangermann S, Lobello C, Oppelt J, Anagnostopoulos I, Hielscher T, Pervez S, Klapper W, Zammarchi F, Silva DA, Garcia KC, Baker D, Janz M, Schleussner N, Fend F, Pospíšilová Š, Janiková A, Wallwitz J, Stoiber D, Simonitsch-Klupp I, Cerroni L, Pileri S, de Leval L, Sibon D, Fataccioli V, Gaulard P, Assaf C, Knörr F, Damm-Welk C, Woessmann W, Turner SD, Look AT, Mathas S, Kenner L, Merkel O
Nat Commun 2021 Sep 22;12(1):5577. doi: 10.1038/s41467-021-25379-9. PMID: 34552066Free PMC Article
Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous.
Xing X, Feldman AL
Adv Anat Pathol 2015 Jan;22(1):29-49. doi: 10.1097/PAP.0000000000000047. PMID: 25461779
Anaplastic large cell lymphoma and breast implants: a systematic review.
Kim B, Roth C, Chung KC, Young VL, van Busum K, Schnyer C, Mattke S
Plast Reconstr Surg 2011 Jun;127(6):2141-2150. doi: 10.1097/PRS.0b013e3182172418. PMID: 21358562

Clinical prediction guides

Spindle-cell (Sarcomatoid) Variant of Cutaneous Anaplastic Large-cell Lymphoma (C-ALCL): An Unusual Mimicker of Cutaneous Malignant Mesenchymal Tumors-A Series of 11 Cases.
Gru AA, Bhagat G, Subtil A, Raghavan SS, Pulitzer M, Chung C, Sangueza M, Plaza JA
Am J Surg Pathol 2021 Jun 1;45(6):796-802. doi: 10.1097/PAS.0000000000001623. PMID: 33234878Free PMC Article
Synchronous diagnosis of anaplastic large cell lymphoma and multiple myeloma in a patient: A case report.
Shi X, Wu J, Jiang Q, Zhang S, Chen W, Yu X, Liu Y, Chen M, Peng J, Li T, Zhu Y, Xi X
Medicine (Baltimore) 2020 Oct 30;99(44):e22931. doi: 10.1097/MD.0000000000022931. PMID: 33126356Free PMC Article
Systemic, primary cutaneous, and breast implant-associated ALK-negative anaplastic large-cell lymphomas present similar biologic features despite distinct clinical behavior.
Gerbe A, Alame M, Dereure O, Gonzalez S, Durand L, Tempier A, De Oliveira L, Tourneret A, Costes-Martineau V, Cacheux V, Szablewski V
Virchows Arch 2019 Aug;475(2):163-174. Epub 2019 Apr 6 doi: 10.1007/s00428-019-02570-4. PMID: 30953147
Anaplastic Large Cell Lymphoma: Contemporary Concepts and Optimal Management.
Shustov A, Soma L
Cancer Treat Res 2019;176:127-144. doi: 10.1007/978-3-319-99716-2_6. PMID: 30596216
Primary CNS T-cell Lymphomas: A Clinical, Morphologic, Immunophenotypic, and Molecular Analysis.
Menon MP, Nicolae A, Meeker H, Raffeld M, Xi L, Jegalian AG, Miller DC, Pittaluga S, Jaffe ES
Am J Surg Pathol 2015 Dec;39(12):1719-1729. doi: 10.1097/PAS.0000000000000503. PMID: 26379152Free PMC Article

Recent systematic reviews

Anaplastic large cell lymphoma and breast implants: a systematic review.
Kim B, Roth C, Chung KC, Young VL, van Busum K, Schnyer C, Mattke S
Plast Reconstr Surg 2011 Jun;127(6):2141-2150. doi: 10.1097/PRS.0b013e3182172418. PMID: 21358562

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