BACKGROUND AND OBJECTIVES: Recent reports suggest that CD4(+)/CD56(+)/lineage(-) hematopoietic neoplasias are aggressive types of malignancies involving lymphoplasmacytoid/DC2 dendritic cells (DC). Here, we report on the incidence of DC malignancies and their clinical, biological, phenotypic and cytogenetic characteristics. DESIGN AND METHODS: From a large series of 392 patients with acute myeloblastic leukemia (AML) and 739 with non-Hodgkin's lymphoma (NHL), five cases (three presenting as acute leukemia and two as NHL) showed clinical, morphologic and phenotypic features compatible with a DC malignancy. RESULTS: The overall incidence of DC malignancies among all AML and NHL cases was 0.76% and 0.27%, respectively. At presentation, these patients displayed cutaneous nodules, splenomegaly and lymph node involvement with variable levels of peripheral blood (PB) and/or bone marrow (BM) infiltration in association with anemia and thrombocytopenia. Cytologic studies showed immature appearing blast cells with negative cytochemistry reactions for both myeloperoxidase and esterases. A highly suggestive DC phenotype based on co-expression of CD123(hi)/HLADR(+)/lin(-)/CD56(+)/CD45(dim) associated with a germline configuration of both the IgH and TCRgamma genes was found in all except one patient who was CD56(-). Expression of other markers compatible with a DC origin was found in all cases. INTERPRETATION AND CONCLUSIONS: We show that DC-derived malignancies can present as either cutaneous lymphoma or acute leukemia, although their incidence is extremely low (<< >1%). While most of these DC neoplasias probably correspond to the malignant counterpart of DC2/lymphoplasmacytoid DC, neoplasias of myeloid DC might also exist, chemotherapy followed by consolidation with ASCT is apparently the most effective strategy for achieving a durable remission in these individuals.
BACKGROUND AND OBJECTIVES: Recent reports suggest that CD4(+)/CD56(+)/lineage(-) hematopoietic neoplasias are aggressive types of malignancies involving lymphoplasmacytoid/DC2 dendritic cells (DC). Here, we report on the incidence of DC malignancies and their clinical, biological, phenotypic and cytogenetic characteristics. DESIGN AND METHODS: From a large series of 392 patients with acute myeloblastic leukemia (AML) and 739 with non-Hodgkin's lymphoma (NHL), five cases (three presenting as acute leukemia and two as NHL) showed clinical, morphologic and phenotypic features compatible with a DC malignancy. RESULTS: The overall incidence of DC malignancies among all AML and NHL cases was 0.76% and 0.27%, respectively. At presentation, these patients displayed cutaneous nodules, splenomegaly and lymph node involvement with variable levels of peripheral blood (PB) and/or bone marrow (BM) infiltration in association with anemia and thrombocytopenia. Cytologic studies showed immature appearing blast cells with negative cytochemistry reactions for both myeloperoxidase and esterases. A highly suggestive DC phenotype based on co-expression of CD123(hi)/HLADR(+)/lin(-)/CD56(+)/CD45(dim) associated with a germline configuration of both the IgH and TCRgamma genes was found in all except one patient who was CD56(-). Expression of other markers compatible with a DC origin was found in all cases. INTERPRETATION AND CONCLUSIONS: We show that DC-derived malignancies can present as either cutaneous lymphoma or acute leukemia, although their incidence is extremely low (<< >1%). While most of these DC neoplasias probably correspond to the malignant counterpart of DC2/lymphoplasmacytoid DC, neoplasias of myeloid DC might also exist, chemotherapy followed by consolidation with ASCT is apparently the most effective strategy for achieving a durable remission in these individuals.
Authors: J J M van Dongen; L Lhermitte; S Böttcher; J Almeida; V H J van der Velden; J Flores-Montero; A Rawstron; V Asnafi; Q Lécrevisse; P Lucio; E Mejstrikova; T Szczepański; T Kalina; R de Tute; M Brüggemann; L Sedek; M Cullen; A W Langerak; A Mendonça; E Macintyre; M Martin-Ayuso; O Hrusak; M B Vidriales; A Orfao Journal: Leukemia Date: 2012-05-03 Impact factor: 11.528
Authors: Sarah M Kranick; Priscila H Goncalves; Maryalice Stetler-Stevenson; Karen Aleman; Mark N Polizzotto; Richard F Little; Robert Yarchoan; Thomas S Uldrick Journal: Haematologica Date: 2014-10-10 Impact factor: 9.941