RESUMO
Plasmacytoid dendritic cell neoplasms are aggressive and rare hematologic malignancies characterized by clonal expansion of plasmacytoid dendritic cells with frequent cutaneous involvement. The pathogenesis is not well established, and it shows enhanced expression of CD56, CD4 and CD123 detected by flow cytometry and immunohistochemistry. We report a case report of this rare disease in a hispanic child with complete remission after using a protocol for high-risk acute lymphoblastic leukemia.
RESUMO
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy of the skin and hematopoietic system. There are few pediatric cases reported in the literature. Management of primary cutaneous BPDCN is challenging because, despite an apparently indolent clinical presentation, rapid dissemination with high mortality can occur. We describe a child with isolated cutaneous involvement who had a good response to chemotherapy as first-line treatment of BPDCN.
Assuntos
Neoplasias Hematológicas , Neoplasias Cutâneas , Criança , Células Dendríticas , Diagnóstico Diferencial , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/terapia , Humanos , Pele , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológicoRESUMO
RESUMEN La neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una malignidad hematológica poco frecuente y generalmente agresiva, por lo cual se requiere su reconocimiento precoz. A continuación, se describe el curso clínico prolongado de un paciente masculino de 60 años con NBCDP procedente de Venezuela, en cuyos hallazgos más relevantes destacó la presencia de lesiones cutáneas, organomegalias, infiltración de la médula ósea y del sistema nervioso central. Posterior al diagnóstico se indicó quimioterapia sistémica, no obstante, el paciente falleció por complicaciones respiratorias durante la fase de inducción del tratamiento. En esta enfermedad es necesario establecer el diagnóstico diferencial con trastornos linfoproliferativos, leucemias linfoides y mieloides agudas, constituyendo el análisis morfológico de las células neoplásicas un aspecto importante para una adecuada orientación diagnóstica.
ABSTRACT Blastic plasmacytoid dendritic cell blast neoplasm (BPDCN) is a rare and generally aggressive hematologic malignancy, requiring early recognition. Below is a description of the prolonged clinical course of a 60-year-old male patient with BPDCN from Venezuela, whose most relevant findings highlighted the presence of skin lesions, organomegaly, infiltration of the bone marrow and central nervous system. Systemic chemotherapy was prescribed after diagnosis; however, the patient died of respiratory complications during the induction phase of treatment. In this disease, it is necessary to establish the differential diagnosis with lymphoproliferative disorders, acute lymphoid and myeloid leukemias. The morphological analysis of neoplastic cells is, thus, an important aspect toward proper diagnostic guidance.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/diagnóstico , Células Dendríticas/patologia , Leucemia Mieloide Aguda/diagnóstico , Neoplasias Cutâneas/patologia , Leucemia Mieloide Aguda/patologia , Diagnóstico Diferencial , Transtornos Linfoproliferativos/diagnósticoRESUMO
We present 2 cases of blastic plasmacytoid dendritic cell neoplasm (BPDCN) showing unusual histological features. One patient, a 73-year-old male, presented with a nonpruritic macular erythema of the skin on the anterior and posterior chest wall, the biopsy of which was originally diagnosed as malignant melanoma. The neoplastic cells were negative for S100 and HMB45 and strongly positive for CD45, CD4, CD56, and CD123. The final diagnosis was a BPDCN associated with abundant melanin pigment and numerous melanophages. The second patient was a 73-year-old male with a 5-month history of small, slowly enlarging, bruise-like plaques on his limbs and chest. Histologic examination of the skin biopsy revealed an atypical cellular/myxoid infiltrate with numerous macrophages, which was originally diagnosed as consistent with lepromatous leprosy. The atypical cells were immersed in an alcian blue-positive myxoid matrix at pH 2.5. The Fite-Faraco stain was negative. Positive immunoreactivity was demonstrated for CD4, CD56, and CD123. Based on the histopathology and immunohistochemistry findings, a diagnosis of BPDCN with prominent myxoid matrix was rendered.
Assuntos
Células Dendríticas/patologia , Erros de Diagnóstico , Neoplasias Hematológicas/diagnóstico , Hanseníase Virchowiana/diagnóstico , Melanoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Idoso , Biópsia , Citodiagnóstico , Humanos , MasculinoRESUMO
Los carcinomas basocelulares gigantes (CBCG) son muy poco frecuentes. Se los puede definir como aquellos mayores de 5 cm de diámetro. La mayoría de estos pacientes no refiere antecedentes deexposición crónica al sol ni presentan otros factores de riesgo conocidos. Existe en cambio una relación estrecha con situaciones de negligencia y tratamientos inadecuados. Se los encuentra frecuentemente en el tronco. Las lesiones mayores de 10 cm de diámetro tienen un alto riesgo de generar metástasis y en consecuencia producir la muerte. Presentamos una paciente de 92 años con dos masas tumorales en área malar y mejilla derecha que fue tratada quirúrgicamente, con buena evolución hasta los dos años de seguimiento posterior.
CD4+ / CD56 malignancy is an extremely rare hematological neoplasm, which was recentlyshown to correspond to the so-called type 2 dendritic cell or plasmacytoid dendritic cell. Clinicalpresentation typically correspond to cutaneous nodules o tumors associated with lymphadenopathyor spleen enlargement or both, cytopenias and circulating malignant cells. The prognosis is rapidlyfatal in the absence of chemotherapy. Bone marrow transplantation is the best option available. Wepresent two new cases of this disease.