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RESUMEN La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una malignidad hematológica poco frecuente y generalmente agresiva, por lo cual se requiere su reconocimiento precoz. A continuación, se describe el curso clínico de una paciente de 72 años con NCDPB, en cuyos hallazgos más relevantes destacó la presencia de lesiones cutáneas, organomegalias, infiltración de la médula ósea. Posterior al diagnóstico se indicó quimioterapia sistémica, con buena respuesta, no obstante, a los 2 meses presentó recidiva de lesiones y la paciente falleció. 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 neoplasia (BCDPN) is a rare and generally aggressive hematological malignancy, which requires early recognition. Below, the clinical course of a 72-year-old patient with NCDPB is described, whose most relevant findings included the presence of skin lesions, organomegaly, and bone marrow infiltration. After the diagnosis, systemic chemotherapy was indicated, with a good response; however, after 2 months the lesions recurred and the patient died. In this disease it is necessary to establish the differential diagnosis with lymphoproliferative disorders, lymphoid and acute myeloid leukemias, with the morphological analysis of the neoplastic cells being an important aspect for adequate diagnostic guidance.
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ABSTRACT Introduction and hypothesis: Umbilical cord blood (UCB) is an alternative source of hematopoietic stem cells for allogeneic hematopoietic stem cell transplantation in the absence of a compatible donor. The UCB transplantation has a lower incidence of chronic graft versus host disease (GvHD), but is associated with slower engraftment and slower immune reconstitution, compared to other sources. Dendritic cells (DCs) and Natural Killer cells (NKs) play a central role in the development of GvHD and the graft versus leukemia (GvL) effect, as well as in the control of infectious complications. Method: We quantified by multiparametric flow cytometry monocytes, lymphocytes, NK cells, and DCs, including their subsets, in UCB samples from 54 healthy newborns and peripheral blood (PB) from 25 healthy adult volunteers. Results: In the UCB samples, there were higher counts of NK cells 56bright16- (median 0.024 × 109/L), compared to the PB samples (0.012 × 109/L, p < 0.0001), NK 56dim16bright (median 0.446 × 109/L vs. 0.259 × 109/L for PB samples, p = 0.001) and plasmacytoid dendritic cells (pDCs, median 0.008 × 109/L for UCB samples vs. 0.006 × 109/L for PB samples, p = 0.03). Moreover, non-classic monocyte counts were lower in UCB than in PB (median 0.024 × 109/L vs. 0.051 × 109/L, respectively, p < 0.0001). Conclusion: In conclusion, there were higher counts of NK cells and pDCs and lower counts of non-classic monocytes in UCB than in PB from healthy individuals. These findings might explain the lower incidence and severity of chronic GvHD, although maintaining the GvL effect, in UCB transplant recipients, compared to other stem cell sources.
Subject(s)
Fetal BloodABSTRACT
INTRODUCTION AND HYPOTHESIS: Umbilical cord blood (UCB) is an alternative source of hematopoietic stem cells for allogeneic hematopoietic stem cell transplantation in the absence of a compatible donor. The UCB transplantation has a lower incidence of chronic graft versus host disease (GvHD), but is associated with slower engraftment and slower immune reconstitution, compared to other sources. Dendritic cells (DCs) and Natural Killer cells (NKs) play a central role in the development of GvHD and the graft versus leukemia (GvL) effect, as well as in the control of infectious complications. METHOD: We quantified by multiparametric flow cytometry monocytes, lymphocytes, NK cells, and DCs, including their subsets, in UCB samples from 54 healthy newborns and peripheral blood (PB) from 25 healthy adult volunteers. RESULTS: In the UCB samples, there were higher counts of NK cells 56bright16- (median 0.024 × 109/L), compared to the PB samples (0.012 × 109/L, p < 0.0001), NK 56dim16bright (median 0.446 × 109/L vs. 0.259 × 109/L for PB samples, p = 0.001) and plasmacytoid dendritic cells (pDCs, median 0.008 × 109/L for UCB samples vs. 0.006 × 109/L for PB samples, p = 0.03). Moreover, non-classic monocyte counts were lower in UCB than in PB (median 0.024 × 109/L vs. 0.051 × 109/L, respectively, p < 0.0001). CONCLUSION: In conclusion, there were higher counts of NK cells and pDCs and lower counts of non-classic monocytes in UCB than in PB from healthy individuals. These findings might explain the lower incidence and severity of chronic GvHD, although maintaining the GvL effect, in UCB transplant recipients, compared to other stem cell sources.
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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.
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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.
Subject(s)
Hematologic Neoplasms , Skin Neoplasms , Child , Dendritic Cells , Diagnosis, Differential , Hematologic Neoplasms/diagnosis , Hematologic Neoplasms/therapy , Humans , Skin , Skin Neoplasms/diagnosis , Skin Neoplasms/drug therapyABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Skin Neoplasms/diagnosis , Dendritic Cells/pathology , Leukemia, Myeloid, Acute/diagnosis , Skin Neoplasms/pathology , Leukemia, Myeloid, Acute/pathology , Diagnosis, Differential , Lymphoproliferative Disorders/diagnosisABSTRACT
During dengue virus (DENV) infection, a blockage of secretion of cytokines such as tumor necrosis factor (TNF)-α and members of the interferon (IFN) family has been described in vitro. We evaluated the functionality of monocytes as well as dendritic, B and T cells isolated from children with mild and severe dengue. Compared with those of healthy children, stimulated monocytes, CD4+ T cells and dendritic cells from children with dengue had lower production of proinflammatory cytokines. The interferon axis was dramatically modulated by infection as plasmacytoid dendritic cells (pDCs) and CD4+ T cells had low production of IFN-α and IFN-γ, respectively; plasma levels of IFN-α and IFN-γ were lower in severely ill children, suggesting a protective role. Patients with antigenemia had the highest levels of IFN-α in plasma but the lowest frequency of IFN-α-producing pDCs, suggesting that DENV infection stimulates a systemic type I IFN response but affects the pDCs function.
Subject(s)
Dengue Virus/physiology , Dengue/immunology , Leukocytes, Mononuclear/immunology , Adolescent , Child , Child, Preschool , Dengue/virology , Dengue Virus/genetics , Female , Humans , Infant , Interferon-alpha/immunology , Male , Monocytes/immunology , Pediatrics/statistics & numerical data , Tumor Necrosis Factor-alpha/immunologyABSTRACT
La neoplasia de células blásticas plasmocitoides dendríticas esun linfoma cutáneo poco frecuente y de mal pronóstico, que característicamenteexpresa antígenos CD4 y CD56. Clínicamentepresenta placas o nódulos de coloración violácea, únicos o múltiples.El diagnóstico se confirma con el estudio anatomopatológicoque evidencia células linfoblástica y fenotipo CD4+ y CD56+, conausencia de marcadores para células mieloides, linfoides B y T ycélulas NK. Presentamos el reporte de un caso de un paciente desexo masculino de 65 años de edad y una revisión de la literatura.
Blastic plasmacytoid dendritic cell neoplasm is a rare andhighly aggressive cutaneous lymphoma that characteristicallyexpress CD4 and CD56 antigens. Clinically presents violet colouredplaques or nodules, in a unique or multiple presentation.The diagnosis is confirmed with the anatomophatologic studythat evidence linfoblastic cells with CD4+ and CD56+ phenotype,and no mieloids, linfoids B and T and natural killers cellsmarkers. We present a case report of a 65-years-old male anda literature review.
Subject(s)
Male , Humans , Aged , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Hematologic Neoplasms/diagnosis , Hematologic Neoplasms/pathology , Skin Neoplasms , Hematologic Neoplasms , Dendritic Cells/pathology , Fatal OutcomeABSTRACT
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.
Subject(s)
Dendritic Cells/pathology , Diagnostic Errors , Hematologic Neoplasms/diagnosis , Leprosy, Lepromatous/diagnosis , Melanoma/diagnosis , Skin Neoplasms/diagnosis , Aged , Biopsy , Cytodiagnosis , Humans , MaleABSTRACT
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.
Subject(s)
Humans , Female , Aged , Carcinoma, Basal Cell/surgery , Carcinoma, Basal Cell/diagnosis , Carcinoma, Basal Cell/pathology , Skin Neoplasms/surgery , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Facial Neoplasms/pathology , Skin Ulcer/surgery , Skin Ulcer/pathologyABSTRACT
La neoplasia hematodérmica CD4+ CD56+ con fenotipo de célula dendrítica plasmocitoide es una rara y agresiva neoplasia recientemente reconocida por la WHO-EORTC classification. Afecta adultos de edad media y ancianos, siendo muy pocos los casos descriptos en niños. Presentamos el caso de una niña de 12 años con grave retraso mental, estigmas genéticos y múltiples lesiones cutáneas localizadas en miembros inferiores y superiores. Histológicamente se observó un infiltrado dérmico difuso de células pequeñas y medianas con expresión de CD4, CD56, CD43 y S100 así como de marcadores dendríticos plasmocitoides: CD 123 y BDCA-2 confirmados por citometría de flujo, sin compromiso de sangre periférica ni médula ósea. Cumpliendo dos semanas de tratamiento para leucemia linfoblástica aguda evolucionó con remisión clínica de las lesiones cutaneas.
Hematodermic CD4+ CD56+ neoplasm with plasmacytoid dendritic cell phenotype is a rare and aggressive neoplasm recently recognized by the WHO-EORTC classification. It generally appears in elderly adults, exceptionally in childhood. We present a 12-year-old girl with severe mental retardation, genetic clinical features and multiple nodular cutaneous lesions on legs and arms. Histologically the nodules showed diffuse dermal infiltrate of medium and small cells and expression of CD4, CD56, CD43, S100 and plasmacytoid dendritic markers: CD123, BDCA-2 under flow cytometry study. Peripheral blood and bone marrow were not involved. Clinical remission of cutaneous lesions was observed after two weeks of acute lymphoblastic leukemia therapy.