Primary cytotoxic T-cell lymphomas harbor recurrent targetable alterations in the JAK-STAT pathway

Author:

Lee Katie12ORCID,Evans Mark G.3ORCID,Yang Lei4,Ng Spencer1ORCID,Snowden Caroline12,Khodadoust Michael51ORCID,Brown Ryanne A.16ORCID,Trum Nicholas A.7ORCID,Querfeld Christiane7ORCID,Doan Linda T.8,Song Jinming9,Zhang Hailing9,Gru Alejandro A.10,Wood Gary S.11,Wada David A.112,Shanmugam Vignesh3,Haun Paul L.13,Aster Jon C.3,Duncan Lyn M.14,Guitart Joan1,Weinstock David M.4ORCID,Nardi Valentina14,Choi Jaehyuk121516ORCID

Affiliation:

1. Department of Dermatology and

2. Department of Biochemistry and Molecular Genetics, Northwestern University Feinberg School of Medicine, Chicago, IL;

3. Department of Pathology, Brigham and Women’s Hospital and

4. Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;

5. Division of Oncology, Department of Medicine

6. Department of Pathology, Stanford University School of Medicine, Stanford, CA;

7. Department of Dermatology, City of Hope National Medical Center, Duarte, CA;

8. Department of Dermatology, University of California, Irvine, Irvine, CA;

9. Department of Pathology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL;

10. Department of Pathology, University of Virginia, Charlottesville, VA;

11. Department of Dermatology, University of Wisconsin, Madison, WI;

12. Huntsman Cancer Institute, University of Utah Health Sciences Center, Salt Lake City, UT;

13. Penn Cutaneous Lymphoma Program, Department of Dermatology, Perelman Center for Advanced Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA;

14. Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA;

15. Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL; and

16. Center for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL

Abstract

Primary cytotoxic T cell lymphomas (CTCLs) are a rare subset of aggressive, poor-prognosis T-cell lymphomas targeting the skin; they include primary cutaneous γδT-cell lymphoma, primary cutaneous VD8+ aggressive epidermotropic T cell lymphoma (PCAETCL), and cytotoxic CTCL not otherwise specified. Lee et al report that all 3 subsets have JAK-STAT activation, but PCAETCL uniquely carries JAK2 gene fusions that may render them especially susceptible to JAK inhibitors.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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