Anaplastic large cell lymphoma: a clinicopathologic analysis

Abstract

The clinicopathologic features of anaplastic large cell lymphoma (ALCL) are reviewed. ALCL is a heterogeneous group of tumours, and histologic examination alone is not adequate in providing useful prognostic information. However, using a combination of clinical, phenotypic, and genotypic features, several distinct clinicopathologic entities have been identified. A subset of ALCL as presently defined is characterized by a balanced translocation, t(2;5)(p23;q35), resulting in a novel fusion protein (NPM-ALK) that can be readily detected by immunohistochemical methods using antibodies against the ALK protein. Detection of ALK protein, along with other methods for demonstrating the t(2;5), has assisted in identifying a distinct biologic entity within the heterogeneous group of ALCL with significant prognostic implications. It is important to separate these from cases of ALK-negative ALCL, which have a poorer prognosis, and cases of primary cutaneous ALCL, which have an excellent prognosis.

Primary Systemic Anaplastic Large Cell Lymphoma in Korean Adults: 11 Years' Experience at Asan Medical Center

“…The main epidemiologic feature was a relatively higher incidence in the fourth (28%) and fifth (28%) decades of life and a median age of 39 years, a finding in close agreement with previous reports,16,17 but in disagreement with other earlier studies that showed a bimodal age distribution with a larger peak in the second and third decades and a smaller peak in the sixth and seventh decades 9. However, the distribution of elderly patients could be underestimated because elderly patients are generally reluctant to receive chemotherapy in Korea.…”

CD30+ TIA-1+ ALK+ Anaplastic Large Cell Lymphoma: Studies of Three Cases by Flow Cytometry Analysis, Immunohistochemistry and Electron Microscopy

“…Recently, the ALK-positive ALCL has been identified as a single entity, which shows distinctive clinical, pathological, phenotypical and molecular features [7,8,18,26,27,29]. About 80-85% of ALK-positive ALCL express the NPM-ALK fusion protein generated by the t(2;5) translocation, whereas 15-20% of the cases express ALK variant fusion proteins (TPM3-ALK, TFG-ALK, ATIC-ALK, Clathrin-ALK, and Moesin-ALK) created by chromosomal translocations in which ALK fuses to a gene partner other than NPM.…”

Translocations involving anaplastic lymphoma kinase (ALK)

“…This achievement was enhanced by the availability of speci®c antibodies that recognize ALK fusion proteins in para n-embedded lymphoma tissues. Several excellent recent reviews have summarized the histopathological and molecular ®ndings of ALCL and their use in the classi®cation of this lymphoma entity (Anagnostopoulos and Benharroch et al, 1998;Drexler et al, 2000;Foss et al, 2000;Gogusev and Nezelof, 1998;Kadin and Morris, 1998;Ladanyi, 1997;Morris et al, 2001;Shiota and Mori, 1996;Skinnider et al, 1999;Stein et al, 2000). This review will focus on the molecular function and signal transduction pathways activated by ALK fusion oncogenes, with recent advances and possible clinical implications to be discussed.…”

“…Keywords: ALK; NPM ± ALK; variant ALK fusions; ALCL; IMT; pleiotrophin ALCL ALCL belongs to the group of high-grade NHLs and typically presents as an aggressive systemic disease, with or without extranodal involvement (for reviews see Kadin, 1997;Kinney and Kadin, 1999;Skinnider et al, 1999;Stein et al, 2000). ALCL tumor cells are characterized by the expression of the CD30/Ki-1 antigen.…”