Rare subtypes of peripheral T-cell lymphoma (PTCL) including enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and hepatosplenic T-cell lymphoma (HSTCL) are underrepresented in most registry and clinical studies. Most of the literature is obtained from small case series, single-institution retrospective studies and subgroup analyses of the largest studies with few recent and ongoing exceptions. While the pathogenesis and biology of these entities have yet to be fully elucidated, global efforts by the scientific community have started to shed some light on the most frequently deregulated pathways. In this review, we highlight the most pertinent clinical and pathologic features of rare subtypes of PTCL including EATL/MEITL, SPTCL and HSTCL. We also summarize the results of recent developments identifying potential targets for novel therapeutic strategies based on molecular studies. Finally, we highlight the underrepresentation of these rare subtypes in most clinical trials, making evidence-based therapeutic decisions extremely challenging.
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Review Article|
April 24, 2024
Current and upcoming treatment approaches to uncommon subtypes of PTCL (EATL/MEITL, SPTCL, HSTCL)
Enrica Marchi,
University of Virginia Cancer Center, Charlottesville, Virginia, United States
* Corresponding Author; email: em5yt@uvahealth.org
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Jeffrey W Craig,
Jeffrey W Craig
University of Virginia Health System, Charlottesville, Virginia, United States
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Matko Kalac
Matko Kalac
University of California, Irvine, Orange, California, United States
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Blood blood.2023021788.
Article history
Submitted:
January 25, 2024
Revision Received:
April 19, 2024
Accepted:
April 19, 2024
Citation
Enrica Marchi, Jeffrey W Craig, Matko Kalac; Current and upcoming treatment approaches to uncommon subtypes of PTCL (EATL/MEITL, SPTCL, HSTCL). Blood 2024; blood.2023021788. doi: https://doi.org/10.1182/blood.2023021788
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