title: Haematodermic CD4+CD56+ neoplasm: complete remission after methotrexate-asparaginase treatment.
abstract: Haematodermic neoplasm is a recently recognized condition, characterized by tumour cells expressing CD4, CD56 and CD123. This phenotype is strongly suggestive of a plasmacytoid dendritic cell origin. This haematopoietic malignancy is a distinct clinicopathological condition with frequent skin involvement, an evolution toward leukaemia and a rapidly aggressive course. We report the case of a 64-year-old woman who presented with a haematodermic CD4+CD56+CD123+ neoplasm affecting the left cheek; the initial staging was otherwise negative. Despite this early stage of the disease, aggressive treatment including methotrexate-asparaginase and local radiotherapy was proposed as first-line therapy. Complete clinical remission was rapidly reached and the patient was still alive after > 30 months of follow-up. To date there is no consensus on the first-line treatment for such patients but intensive treatment is probably needed immediately even in cases of localized disease. The response obtained with CHOP (cyclophosphamide, doxyrubicin, vincristine, prednisone) or CHOP-like chemotherapy regimens is disappointing. Other regimens, such as those used in acute leukaemia, may improve the outcome of these patients.
authors.affiliation: Department of Dermatology, Claude Bernard University, Lyon Civil Hospices, Hotel-Dieu Hospital, Lyon, France.
authors.forename: J
authors.initials: J
authors.lastname: Fontaine
authors.forename: L
authors.initials: L
authors.lastname: Thomas
authors.forename: B
authors.initials: B
authors.lastname: Balme
authors.forename: S
authors.initials: S
authors.lastname: Ronger-Savle
authors.forename: C
authors.initials: C
authors.lastname: Traullé
authors.forename: T
authors.initials: T
authors.lastname: Petrella
authors.forename: S
authors.initials: S
authors.lastname: Dalle
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001215:Asparaginase / Q000008:administration & dosage; D005153:Facial Neoplasms / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy* / Q000473:pathology; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology
chemical_list: D001215:Asparaginase; D008727:Methotrexate