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Mediastinal Anaplastic Large-Cell Lymphoma

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Pathology of the Pleura and Mediastinum

Part of the book series: Encyclopedia of Pathology ((EP))

Synonyms

Ki-1 lymphoma; Ki-1-positive large-cell lymphoma; Lymphoma; Large-cell anaplastic, CD30-positive; Malignant histiocytosis

Definition

Anaplastic large-cell lymphoma (ALCL) is a mature T-cell neoplasm characterized by strong CD30 expression and the presence of “hallmark cells,” which are usually large atypical cells with kidney- or horseshoe-shaped nuclei, abundant cytoplasm, and characteristic perinuclear (Golgi) clearing. ALCL also tends to have a cohesive growth pattern and a propensity for lymph node sinusoidal distribution. The current 2008 WHO classification of hematolymphoid neoplasms distinguishes an ALK-positive and an ALK-negative variant of ALCL. ALK-positive ALCL is characterized by translocations involving the ALK gene, resulting in overexpression of novel chimeric ALK proteins. ALK-negative ALCL is morphologically and immunophenotypically similar to ALK-positive ALCL but lacks ALK gene rearrangements and ALK protein expression. It is a provisional entity in the...

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References and Further Reading

  • Armitage, J. O. (2012). The aggressive peripheral T-cell lymphomas: 2012 update on diagnosis, risk stratification, and management. American Journal of Hematology, 87(5), 511–519.

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  • Falini, B. (2001). Anaplastic large cell lymphoma: Pathological, molecular and clinical features. British Journal of Haematology, 114(4), 741–760.

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  • Medeiros, L. J., & Elenitoba-Johnson, K. S. (2007). Anaplastic large cell lymphoma. American Journal of Clinical Pathology, 127(5), 707–722.

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  • Merkel, O., Hamacher, F., Sifft, E., Kenner, L., & Greil, R. (2012). Novel therapeutic options in anaplastic large cell lymphoma: Molecular targets and immunological tools. Molecular Cancer Therapeutics, 10(7), 1127–1136.

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  • Savage, K. J., Harris, N. L., Vose, J. M., Ullrich, F., Jaffe, E. S., Connors, J. M., et al. (2008). 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. Blood, 111(12), 5496–5504.

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Correspondence to Horatiu Olteanu M.D., Ph.D. .

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Olteanu, H. (2018). Mediastinal Anaplastic Large-Cell Lymphoma. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_34

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  • DOI: https://doi.org/10.1007/978-3-319-66796-6_34

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-66795-9

  • Online ISBN: 978-3-319-66796-6

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