CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer

Assaf, Chalid; Gellrich, Sylke; Whittaker, Sean; Robson, Alistair; Cerroni, Lorenzo; Massone, Cesare; Kerl, Helmut; Rosé, Christian; Chott, Andreas; Chimenti, Sergio; Hallermann, Christian; Petrella, Tony; Wechsler, Janine; Bagot, Martine; Hummel, Michael; Bullani-Kerl, K.; Bekkenk, Marcel W.; Kempf, Werner; Meijer, Chris J.L.M.; Willemze, Rein; Sterry, Wolfram

doi:10.1136/jcp.2006.042135

Cited by 121 publications

(108 citation statements)

References 43 publications

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“…En el estudio de IHQ, las células neoplásicas típicamente expresan CD4, CD56 y CD123; así como el antígeno sanguíneo de célula dendrítica 2 (BDCA/CD303) y la molécula dependiente de interferón α 4,15 .…”

Section: Discussionunclassified

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

et al. 2017

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Blastic plasmacytoid dendritic cell neoplasm.Report of three cases L a neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una entidad relativamente rara que afecta principalmente pacientes adultos del sexo masculino 1,2 . Su contraparte normal es la célula dendrítica plasmocitoide de origen hematopoyético, la cual expresa el receptor α de la IL-3 (CD123) en su superficie y carece de marcadores de estirpe mieloide y linfoide. Su función biológica consiste principalmente en establecer un mecanismo de regulación entre la respuesta inmunológica innata y específica 3,4 .La mayoría de los casos se manifiestan como una enfermedad cutánea en forma de nódulos y/o placas 5 , con compromiso de la médula ósea y/o sangre periférica 6 .El diagnóstico se establece mediante el estudio histopatológico de las lesiones cutáneas, caracterizado por la proliferación de células neoplásicas de aspecto blástico que infiltran la dermis y en algunos casos el tejido subcutáneo, con expresión de CD4, CD56, CD123, TCL-1 y CD303 4 . El comportamiento biológico es agresivo y muchos pacientes fallecen en un período corto después del diagnóstico 3 .El objetivo de este estudio es informar las características clínico-patológicas de la NBCDP diagnosticados en el Instituto Nacional de Cancerología de México.

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Section: Discussionunclassified

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

et al. 2017

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“…The frequent occurrence of CNS relapses suggests adopting, as induction treatment, intensive acute lymphoblastic leukemia-like regimens with intrathecal prophylaxis [7]. The use of RT has been only occasionally reported [5,11,12], mostly for the treatment of localized lesions or in the consolidation phase.…”

Section: Treatmentmentioning

confidence: 99%

Plasmacytoid Dendritic Cell Leukemia: A Rapidly Evolving Disease Presenting with Skin Lesions Sensitive to Radiotherapy plus Hyperthermia

et al. 2009

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Plasmacytoid dendritic cell leukemia (pDCL) is a rapidly evolving disease, which frequently presents with skin lesions, particularly nodules and plaques with a typical reddish-brown or brown color. Treatment of pDCL is based on multiagent chemotherapy followed by allogeneic hematopoietic stem cell transplant, but skin lesions may be refractory to therapy. Here, we report on a 61-year-old patient affected by pDCL who first presented with multiple cutaneous nodules and plaques on the trunk. Lesions showed an excellent response to radiotherapy plus hyperthermia. Although this treatment did not avoid the systemic evolution of disease, it resolved skin lesions and prevented their relapse, thus representing a therapeutic option to be used in combination with chemotherapy regimens. The case presentation is followed by a general discussion with an emphasis on the diagnosis and treatment of this rare malignancy. The Oncologist 2009;14:1205-1208 CASE PRESENTATIONA 61-year-old white man was referred with a diagnosis of acute monocytic leukemia cutis manifesting with a 3-month history of skin nodules. The patient presented with reddishbrown or brown multiple skin nodules and plaques up to 5 cm in diameter on the trunk and neck. Brown macules were also present on the upper trunk. Lesions were moderately itchy and painful. He had no systemic symptoms. Physical examination revealed axillary lymphadenopathies. Routine blood tests and a peripheral blood smear were negative. The bone marrow aspirate showed low-level involvement (20%) by medium/large-sized agranular blast cells present-

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“…He died 13 months after initial diagnosis during conservative treatment for pulmonary edema and renal failure. 12 . HN has a highly aggressive clinical course with a poor prognosis and a median survival of 14 months 2,13 .…”

Section: Case Reportmentioning

confidence: 99%

CD4-/CD56+/CD123+ Hematodermic Neoplasm Showing Early Liver Metastasis

Choi

Lee

et al. 2010

Ann Dermatol

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Hematodermic neoplasm (HN) is a clinically aggressive neoplasm with a high incidence of cutaneous involvement and a risk of leukemic dissemination. In the recent WHO-EORTC classification, the term blastic natural killer cell lymphoma has been replaced with CD4＋/CD56＋ HN because of its derivation from a plasmacytoid dendritic cell precursor. Cases of HN that completely lack CD4 or CD56 expression, therefore represents a diagnostic problem. A 68-year-old Korean male was diagnosed with CD4−/CD56＋ HN and treated with hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) at initial treatment, and then switched to high dose methotrexate/ cytarabine. His disease relapsed and resulted in death from bone and brain disease 6 months after complete clinical remission, despite diagnostic workups, including a radioisotope liver scan and ultrasound-guided fine needle aspiration biopsy. Further cytogenetic studies such as comparative genomic hybridization could elucidate the genetic mechanisms in the development and progression of lymphomas. We report an unusual case of 'CD4-/CD56+/CD123+ HN' showing early liver metastasis. (Ann Dermatol 22(2) 186∼190, 2010)

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CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer

Abstract: Results show that CD56+ cutaneous lymphoproliferative disorders, with the exception of CD56+ CTCL have a very poor prognosis. It is therefore clinically important to separate CD56+ CTCL from the remaining CD56+ haematological disorders.

Cited by 121 publications

References 43 publications

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

Neoplasia blástica de células dendríticas plasmocitoides. Casos clínicos

Plasmacytoid Dendritic Cell Leukemia: A Rapidly Evolving Disease Presenting with Skin Lesions Sensitive to Radiotherapy plus Hyperthermia

CD4-/CD56+/CD123+ Hematodermic Neoplasm Showing Early Liver Metastasis

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