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Cutaneous T-Cell Lymphoma, a Novel Manifestation of PDGFRA-Rearranged Neoplasm

Behdad, Amir, MD*; Allen, Pamela, MD; Lu, Xinyan, MD*; Zhou, Xiaolong Alan, MD; Guitart, Joan, MD; Chen, Qing, MD, PhD*; Pro, Barbara, MD

The American Journal of Dermatopathology: August 2018 - Volume 40 - Issue 8 - p 610–613
doi: 10.1097/DAD.0000000000001121
Extraordinary Case Report

Abstract: Patients with PDGFRA-rearranged hematopoietic neoplasms typically present with chronic eosinophilic leukemia and rarely with acute myeloid leukemia or T-lymphoblastic lymphoma. However, mature T-cell lymphoma has not been previously associated with PDGFRA aberrations. We report a patient who presented with simultaneous T-lymphoblastic lymphoma, focal myeloid proliferation, and cutaneous cytotoxic T-cell lymphoma refractory to chemotherapy. The presence of myeloid and lymphoid lineages prompted genetic and molecular studies. A PDGFRA rearrangement was identified in all compartments: cutaneous, lymph node, and bone marrow. Treatment with imatinib resulted in an excellent response in cutaneous and systemic disease. We report the first case of a mature cutaneous T-cell lymphoma with PDGFRA rearrangement, expanding the spectrum of neoplasms associated with this genetic abnormality. Our case underscores the great importance of recognizing PDGFRA rearrangement in unusual cases of cutaneous T-cell lymphoma, systemic lymphoid, and myeloid neoplasms. These patients may respond well to tyrosine kinase inhibitors, despite resistance to conventional chemotherapy.

*Department of Pathology, Northwestern University, Chicago, IL;

Division of Hematology/Oncology, Department of Medicine, Northwestern University, Chicago, IL; and

Department of Dermatology, Northwestern University, Chicago, IL.

Correspondence: Amir Behdad, MD, Assistant Professor in Pathology, Division of Hematopathology, Northwestern University Feinberg School of Medicine, Northwestern Memorial Hospital, 251 East Huron, Feinberg 7-210, Chicago, IL 60611 (e-mail:

Q. Chen and B. Pro contributed equally to this article.

The authors declare no funding or conflicts of interest.

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