title: CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate.
abstract: The CD4(+) CD56(+) hematodermic/plasmacytoid dendritic cell tumor is a rare, highly aggressive, systemic neoplasm for which effective therapies have not yet been established. These tumors express CD4, CD56, CD123, and T-cell leukemia/lymphoma (TCL)-1 and are clinically characterized by cutaneous involvement with spread to bone marrow and blood, and poor prognosis with current chemotherapy regimens. We describe a Caucasian woman who presented with plasmacytoid dendritic cell tumor, but an absence of systemic symptoms. Clinically, multiple cutaneous lesions were brown to violaceous firm nodules on the face, arms, and trunk. The patient underwent two courses of cyclophosphamide, Adriamycin, vincristine, and prednisone chemotherapy but relapsed quickly. The investigational agent, pralatrexate (30 mg/m(2)) was given weekly with vitamin B12 and folic acid and resulted in remarkable clinical response with regression of skin tumors. Our observation highlights pralatrexate as a promising therapeutic option for hematodermic/plasmacytoid dendritic cell lymphoma/leukemias.
authors.affiliation: Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas 77030-4009, USA.
authors.forename: Justin J
authors.initials: JJ
authors.lastname: Leitenberger
authors.forename: Cindy N
authors.initials: CN
authors.lastname: Berthelot
authors.forename: Kristel D
authors.initials: KD
authors.lastname: Polder
authors.forename: Barbara
authors.initials: B
authors.lastname: Pro
authors.forename: Peter
authors.initials: P
authors.lastname: McLaughlin
authors.forename: Dan
authors.initials: D
authors.lastname: Jones
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000368:Aged; D000630:Aminopterin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology*; D004317:Doxorubicin / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005492:Folic Acid / Q000627:therapeutic use; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D009364:Neoplasm Recurrence, Local; D049268:Positron-Emission Tomography; D011241:Prednisone / Q000627:therapeutic use; D012878:Skin Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use; D014805:Vitamin B 12 / Q000627:therapeutic use
chemical_list: C418863:10-propargyl-10-deazaaminopterin; D015704:CD4 Antigens; D019002:CD56 Antigen; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D005492:Folic Acid; D000630:Aminopterin; D014805:Vitamin B 12; D011241:Prednisone