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Systemic ALK-negative anaplastic large cell lymphoma: Insights into morphologic, immunophenotypic, genetic and molecular characteristics.
Hum Pathol
October 2024
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX, 77030, USA. Electronic address:
Anaplastic large cell lymphoma (ALCL) is a mature T-cell neoplasm characterized by large pleomorphic cells, often with horseshoe- or kidney-shaped nuclei and abundant cytoplasm (hallmark cells), and uniformly strong CD30 expression. Based on ALK expression or ALK rearrangement, ALCL is further classified into ALK-positive (ALK+) and ALK-negative types. This review focuses on the clinicopathologic, immunophenotypic, cytogenetic and molecular features of systemic ALK-negative ALCL.
View Article and Find Full Text PDFLymphomatoid Papulosis With DUSP22 Rearrangement in a Patient With a Historical Diagnosis of Primary Cutaneous Anaplastic Large Cell Lymphoma.
Cureus
August 2024
Department of Pathology - Hematopathology and Dermatopathology, Creighton University School of Medicine, Omaha, USA.
Article Synopsis
- Lymphomatoid papulosis (LyP) with rearrangement is a rare subtype characterized by two types of T-cells: small- to medium-sized weakly CD30+ cells and medium- to large-sized strongly CD30+ cells.
- This subtype has a generally milder clinical course than primary cutaneous anaplastic large cell lymphoma (pcALCL) and often regresses without treatment.
- A notable case involved an 81-year-old woman who was diagnosed with LyP with rearrangement, having had a previous diagnosis of pcALCL 20 years earlier.
Systemic ALK-negative anaplastic large cell lymphoma with NPM1::TYK2 rearrangement.
J Hematop
December 2024
Department of Pathology, University of Colorado Hospital, University of Colorado School of Medicine, 12605 East 16 Avenue, Aurora, CO, 80045, USA.
Article Synopsis
- Anaplastic large cell lymphoma (ALCL) is a rare type of non-Hodgkin lymphoma, primarily characterized by ALK gene rearrangements, though about 20-50% are ALK negative (ALK- ALCL) and display different genetic changes.
- A unique case of systemic ALK- ALCL involved a genetic fusion (NPM1::TYK2) that complicated diagnosis until next-generation sequencing (NGS) was applied.
- The NGS results revealed clonal rearrangement and a previously unreported NPM1::TYK2 fusion in systemic ALK- ALCL, highlighting its significance and rarity in comparison to other lymphoid conditions.
In ALK-negative anaplastic large cell lymphoma (ALCL), gene rearrangements of and are considered mutually exclusive. The former predicts a favorable prognosis, while the latter is generally unfavorable. We report the first case of ALK-negative ALCL in a leukemic phase with small cell pattern transformation, harboring double-hit rearrangements of the gene by inv(6)(p25q21) and gene by TBL1XR1-TP63 inversion.
View Article and Find Full Text PDFDUSP22-rearranged primary cutaneous CD30-positive T-cell lymphoproliferative disorders and adult T-cell leukemia/lymphoma frequently share the LEF1+/TIA1- immunophenotype.
Hum Pathol
August 2024
Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, 110, Taiwan; Department of Pathology, Taipei Medical University Hospital, Taipei, 110, Taiwan; Department of Pathology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, 704, Taiwan. Electronic address:
Article Synopsis
- DUSP22 rearrangements are genetic changes found in certain types of lymphomas, particularly in systemic and primary cutaneous anaplastic large cell lymphoma, as well as lymphomatoid papulosis, with previous studies mainly highlighting S-ALCL.
- This study examined LEF1/TIA1 expression and MSC mutations in 23 cases of C-ALCL and LyP, finding DUSP22 rearrangements in eight cases and LEF1 expression present in 63% of those cases, while TIA1 was negative across the board.
- The research indicated that the rate of LEF1 expression in DUSP22-rearranged C-ALCL/LyP is lower than that in S-ALCL
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