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2020, Number 1

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Rev Hematol Mex 2020; 21 (1)

Plasmacytoid dendritic blast cell neoplasia

Contreras-Ruiz F, Esparza-Sandoval AC, Aguilar-del Ángel S, Pezina-Cantú C, Méndez-Ramírez N, Gómez-Almaguer D, Gutiérrez-Aguirre CH

Language: Spanish
References: 15
Page: 61-67
PDF size: 268.99 Kb.


ABSTRACT

Background: Plasmacytoid dendritic blast cell neoplasia is a rare disease that accounts for less than 1% of hematologic malignancies. It most often affects elderly men, in the seventh decade of life. The first clinical manifestation is usually with skin lesions that look like plaque or petechiae with subsequent bone marrow and lymph node involvement. Patients present neuromeningeal affectation in 4 to 9% at diagnosis and 17 to 33% in relapses. The leukemic presentation without skin condition is rare, representing less than 0.44% of cases of acute leukemia. The diagnosis requires an extended immunophenotypic analysis, due to overlap with other neoplasms, in addition to clinical and histopathological correlation. There is no standardized therapy; however, better outcomes have been observed in those treated with an acute lymphoid leukemia type scheme and consolidation with allogeneic transplantation. Despite a good initial response, the prognosis is poor, with an average survival of 12 to 14 months.
Clinical case:A 43-year-old male patient, with exclusively leukemic manifestations.
Conclusions: Establishing the diagnosis of plasmacytoid dendritic blast cell neoplasia can be a challenge due to its clinical manifestation and immunophenotype, which can cause confusion with other hematological diseases.


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