Incidence and characteristics of CD4(+)/HLA DRhi dendritic cell malignancies.
Haematologica
; 89(1): 58-69, 2004 Jan.
Article
en En
| MEDLINE
| ID: mdl-14754607
ABSTRACT
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 ANDMETHODS:
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 ANDCONCLUSIONS:
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.
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Colección:
01-internacional
Asunto principal:
Células Dendríticas
/
Linfoma no Hodgkin
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Leucemia Mieloide Aguda
/
Antígenos HLA-DR
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Antígenos CD4
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Epidemiología Molecular
Tipo de estudio:
Incidence_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Aged
/
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Haematologica
Año:
2004
Tipo del documento:
Article
País de afiliación:
España