Cutaneous T-Cell Lymphoma, a Novel Manifestation of PDGFRA-Rearranged Neoplasm.
Am J Dermatopathol
; 40(8): 610-613, 2018 Aug.
Article
em En
| MEDLINE
| ID: mdl-29505472
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma Cutâneo de Células T
/
Receptor alfa de Fator de Crescimento Derivado de Plaquetas
Tipo de estudo:
Prognostic_studies
Limite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
Am J Dermatopathol
Ano de publicação:
2018
Tipo de documento:
Article
País de afiliação:
Israel