In a landscape of complexity, many sALCL cases are misdiagnosed.1
Your role as an expert hematopathologist is critical. 2
Important clues to diagnosis – such as hallmark cells, ALK expression, and importantly, CD30 expression – can help differentiate sALCL from other rare lymphomas, such as PTCL-NOS. 2,3
With your help and expertise accurate diagnosis of sALCL will lead to appropriate treatment decisions for patients. 1,2
Include CD30 expression in differential diagnosis of T-cell lymphomas.3
Systemic anaplastic large cell lymphoma (sALCL) and peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) are both aggressive T-cell lymphomas with some morphological and/or immunophenotypic overlaps, but they are different diseases.1-4
sALCL and PTCL-NOS have distinctive clinical features that can impact patient management.1-4
sALCL is often misdiagnosed as PTCL-NOS, which comprises the largest group of PTCLs.1,3
Accurate differentiation of sALCL (ALK+), sALCL (ALK-), and PTCL-NOS is crucial for determining PROGNOSES and TREATMENT OPTIONS.1
Patients with PTCL-NOS show a poorer prognosis than patients with sALCL. In sALCL, expression of anaplastic lymphoma kinase (ALK) is associated with a better prognosis when treated with standard chemotherapy, while ALK-negative sALCL patients show poorer outcomes.1-3
sALCL and PTCL-NOS share some pathologic features, but are different diseases.1,3 ALK(-) sALCL is often misdiagnosed as PTCL-NOS, which comprises the largest group of PTCLs.1,3 The presence of hallmark cells, CD30 expression, and ALK expression can help differentiate sALCL from PTCL-NOS.1
Differential diagnosis requires adequate immunophenotyping plus molecular and cytogenetic analysis.2
Antigenic markers used to differentiate between ALCL subgroups and other PTCL subtypes include the ALK protein and the paired boxed gene 5 (PAX5) transcription factor; the absence of nuclear PAX5 is one of the most important diagnostic criteria favoring a diagnosis of ALCL (ALK-).2
Adapted with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Non-Hodgkin’s Lymphomas V3.2016. © 2016 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines® and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines, go online to NCCN.org. NATIONAL COMPREHENSIVE CANCER NETWORK®, NCCN®, NCCN GUIDELINES®, and all other NCCN Content are trademarks owned by the National Comprehensive Cancer Network, Inc.
An accurate diagnosis is essential to effective treatment, and remains a significant predictor of outcome.1 In the frontline settings, ALK(+) sALCL has a different recommended therapy, while in other lines, sALCL and PTCL-NOS are generally treated the same way.2,5,6
Several characteristics can help differentiate sALCL from PTCL-NOS. The most prominent include:1,7,11,12
Nicolae A, Pittaluga S, Venkataraman G, et al. Peripheral T-cell lymphomas of follicular T-helper cell derivation with Hodgkin/Reed-Sternberg cells of B-cell lineage: both EBV-positive and EBV-negative variants exist. Am J Surg Pathol. 2013;37(6):816-826.
Geissinger E, Bonzheim I, Krenacs L, et al. Identification of the tumor cells in peripheral T-cell lymphomas by combined polymerase chain reaction-based T-cell receptor beta spectrotyping and immunohistological detection with T-cell receptor beta chain variable region segment-specific antibodies. J Mol Diagn. 2005;7(4):455-464.
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