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: email@example.com).
Q. Chen and B. Pro contributed equally to this article.
The authors declare no funding or conflicts of interest.