Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated...Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+CD56+CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later. Discussion. "Agranular CD4+CD56+hematodermic neoplasm"is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL-3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+CD56+CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.展开更多
Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consul...Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutanous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+CD56+cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred,followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. Comments. The histological presentation of these two patients was very similar withanunusalphenotypeoftumorcellsexpressingCD4,CD56, CD123, but not expressing CD3 and CD20. Some cases have beenpublishedunderthe"termofblasticNKlymphoma"which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting agressive protocols, with bone marrow allograft in the younger patients.展开更多
文摘Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+CD56+CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later. Discussion. "Agranular CD4+CD56+hematodermic neoplasm"is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL-3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+CD56+CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.
文摘Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutanous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+CD56+cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred,followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. Comments. The histological presentation of these two patients was very similar withanunusalphenotypeoftumorcellsexpressingCD4,CD56, CD123, but not expressing CD3 and CD20. Some cases have beenpublishedunderthe"termofblasticNKlymphoma"which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting agressive protocols, with bone marrow allograft in the younger patients.
