title: A rare case of blastic plasmacytoid dendritic cell neoplasm with deletion 7q.31, in the setting of heavy pre-treatment with alkylating chemotherapy.
abstract: Blastic plasmacytoid dendritic cell neoplasm is rare myeloid malignancy clinically characterized by non-pruritic, violaceous and papulo-nodular skin lesions, together with bone marrow and lymph node involvement. Histologically, there is infiltration of dermis by neoplastic mono-nuclear CD4, CD56, CD123 co-expressing cells with epidermal sparing. Most commonly blastic plasmacytoid dendritic cell neoplasm presents as a de-novo condition, and treatment-related blastic plasmacytoid dendritic cell neoplasm is a rare phenomenon. Due to rarity of the disease, there is no established standard of care treatment. Both acute myeloid leukemia and acute lymphoid leukemia type induction regimens have been used for treatment of blastic plasmacytoid dendritic cell neoplasm, with initial response rate of 50%-80%. We present a rare case of therapy-associated blastic plasmacytoid dendritic cell neoplasm in a patient with remote history alkylating agent systemic therapy. A lag period of five to seven years and presence of deletion 7q.31 seen in bone marrow biopsy specimen in our patient are consistent with a likely therapy-associated etiology of his blastic plasmacytoid dendritic cell neoplasm.
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.|2 British Columbia Cancer Agency, Vancouver Island Cancer Center, Victoria, BC, Canada.
authors: Varinder
authors: V
authors: Kaur
authors: 3 St. George's University, True Blue, Grenada.
authors: Arjun
authors: A
authors: Swami
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.
authors: Atrash
authors: A
authors: Shebli
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.
authors: Sara
authors: S
authors: Shalin
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.
authors: Muthu
authors: M
authors: Veeraputhiran
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.
authors: Peter
authors: P
authors: Emanuel
authors: 1 Department of Internal Medicine, Division of Hematology Oncology, University of Arkansas for Medical Science, Little Rock, AR, USA.
authors: Yogesh
authors: Y
authors: Jethava
mesh_terms: D000368:Aged; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003713:Dendritic Cells / Q000473:pathology*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000473:pathology; D008297:Male; D012878:Skin Neoplasms / Q000473:pathology*
chemical_list: D018906:Antineoplastic Agents, Alkylating
keywords: Blastic plasmacytoid dendritic cell neoplasm; blastic plasmacytoid dendritic cell neoplasm; deletion 7q.31; therapy-associated blastic plasmacytoid dendritic cell neoplasm; treatment-associated acute myeloid leukemia