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Haematodermic CD4+CD56+ neoplasm: complete remission after methotrexate–asparaginase treatment
Author(s) -
Fontaine J.,
Thomas L.,
Balme B.,
RongerSavle S.,
Traullé C.,
Petrella T.,
Dalle S.
Publication year - 2009
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.2008.03100.x
Subject(s) - medicine , vincristine , methotrexate , cyclophosphamide , prednisone , neoplasm , asparaginase , chemotherapy , oncology , chop , malignancy , chlorambucil , radiation therapy , complete remission , gastroenterology , leukemia , pathology , lymphoblastic leukemia
Summary 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.