Haematodermic CD4+CD56+ neoplasm: complete remission after methotrexate-asparaginase treatment.
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.
Fontaine
J
J
Department of Dermatology, Claude Bernard University, Lyon Civil Hospices, Hotel-Dieu Hospital, Lyon, France.
Thomas
L
L
Balme
B
B
Ronger-Savle
S
S
Traullé
C
C
Petrella
T
T
Dalle
S
S
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
D001215:Asparaginase; D008727:Methotrexate