[Flow cytometry increases the proportion of valuable samples in cerebrospinal fluid with normal cell count in malignant blood diseases]. / La citometría de flujo aumenta la proporción de muestras valorables en líquido cefalorraquídeo con recuento celular normal en hemopatías malignas.

BACKGROUND: The alteration of cerebrospinal fluid (CSF) in hematologic neoplasms is a poor prognostic marker. The characteristics of CSF are usually analyzed by flow cytometry or cytology. However, paucicellular CSF samples (≤5 cells/dL) can sometimes be considered unsuitable for analysis due to the low number of events. OBJECTIVE: To evaluate the proportion of samples reported as suitable for analysis obtained by cytometry (FCM) and cytology in paucicellular CSF samples. MATERIAL AND METHODS: 169 samples ofpaucicellular CSF corresponding to 115 patients with hematologic neoplasms were selected. The samples were obtained by lumbar puncture in tubes conditioned with EDTA and Transfix®. We characterized the immunophenotype ofCSF samples with an 8-color panel, and 55 samples (32%) were in a small sample tube (SST). In all cases, monocytes were identified by CD14 labeling and T lymphocytes by CD3 labeling. The acquisition was carried out in a FACSCantoII® cytometer, and the analysis was performed using Infinicyt® software. RESULTS: The proportion of samples suitable for analysis was higher in FCM compared to cytology (98% vs 61%, p < 0.000). We identified the presence of T lymphocytes and/or monocytes in most samples (98% and 90%, respectively). In the SST samples, the number of events recorded in low-volume samples (< 1 mL) was lower than in samples with higher volume (140 vs 556, p < 0.001), with a median of identification of 3 cell populations. CONCLUSION: FCM allows the analysis of a higher proportion ofpaucicellular CSF samples than cytology in hematologic neoplasms study.

[Multiple myeloma in systemic lupus erythematosus. Report of two cases]. / Mieloma múltiple en pacientes con lupus eritematoso sistémico: presentación de dos casos.

There is an increased incidence of hematological malignancies, particularly non-Hodgkin lymphoma (NHL) in systemic lupus erythematosus (SLE). In contrast, the concurrence with Multiple Myeloma (MM) is very rare, and the possible pathogenetic mechanisms underlying this association remain unclear. We report two patients who developed MM 15 and four years after being diagnosed as having SLE.

Oxidative damage and nitric oxide synthase induction by surgical uteroplacental circulation restriction in the rabbit fetal heart.

OBJECTIVE: This study investigated the role of oxidative damage and nitric oxide (NO) synthases in the fetal heart using a model of intrauterine growth restriction induced by uteroplacental circulation restriction (UCR). METHODS: New Zealand white rabbits kept under 12-h light cycles, with food and water provided ad libitum, were subjected at day 25 of pregnancy to 40-50% uteroplacental artery ligation. We analyzed the gene expression of enzymes linked to nitric oxide synthesis (iNOS, eNOS, HO-1, and ARG-2), hypoxia inducible factor 1 alpha (HIF-1α), and the state of oxidative stress (protein carbonyl levels) in fetal heart homogenates. Additionally, we studied the histological morphology of the fetal heart. RESULTS: We found that fetal growth restriction was associated with a significant reduction in heart weight but a normal heart/body weight ratio in UCR animals. Hematoxylin and eosin staining showed normal left and right ventricular thickness but increased vessel dilatation with hyperemia in the hearts of the UCR group. We observed HIF-1α, eNOS, p-eNOS, and iNOS induction concomitant with intensified protein carbonyl levels but observed no changes in HO-1 or ARG-2 expression, suggesting increased NO and oxidative stress in the hearts of UCR animals. CONCLUSION: Uteroplacental circulation restriction increased NO-linked enzymes, oxidative damage, and dilated coronary vessels in fetal hearts. © 2017 The Authors. Prenatal Diagnosis published by John Wiley & Sons, Ltd.

[Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: Report of one case]. / Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central: Caso clínico.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 year-old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.

Nitric oxide synthase and changes in oxidative stress levels in embryonic kidney observed in a rabbit model of intrauterine growth restriction.

OBJECTIVE: This work aimed to study the effect of uteroplacental circulation restriction on endothelial kidney damage in a fetal rabbit model. METHODS: New Zealand rabbits were subjected to 40% to 50% of uteroplacental artery ligation at day 25 of pregnancy. After 5 days, surviving fetuses were harvested by cesarean section. The gene and protein expressions of selected enzymes associated with nitric oxide production and oxidative stress were analyzed in fetal kidney homogenates. RESULTS: The placenta weight (6.06 ± 0.27, p < 0.0319) and fetal body (19.90 ± 1.03, p < 0.0001) were significantly reduced in the uteroplacental circulation restriction group. The kidneys from restricted fetuses presented a mild vascular congestion and glomerular capillary congestion, without inflammation or hypertrophy. We found endothelial nitric oxide synthase phosphorylation inhibition (0.23 ± 0.13, p < 0.012) and arginase-2 (0.29 ± 0.14, p < 0.023) protein induction in fetal kidneys of the circulation restriction group. Finally, the kidneys from circulation-restricted fetuses showed increased inducible nitric oxide synthase messenger RNA (mRNA) (2.68 ± 0.24, p < 0.01) and reduced heme oxygenase-1 mRNA (23 ± 1.3, p < 0.003), with increased reactive oxygen species (1.69 ± 0.09, p < 0.001) and nitrotyrosine protein (1.74 ± 0.28, p < 0.003) levels, without changes in Nox mRNA. CONCLUSION: We describe significant deregulation of vascular activity and oxidative damage in kidneys of fetal rabbits that have been exposed to restriction of the uterine circulation. © 2016 John Wiley & Sons, Ltd.

[Efficacy of PCR for the differential diagnosis of tuberculosis in granulomatous lesions of paraffin-embedded formalin fixed tissues]. / Eficacia del diagnóstico diferencial de tuberculosis mediante reacción de polimerasa en cadena en lesiones granulomatosas de tejidos fijados en formalina e incluidos en parafina.

BACKGROUND: Granulomatous lesions occur in tuberculosis (TB), other infections, toxic, allergic, and autoimmune diseases among others. In absence of a an acid-fast bacilli (AFB) confirmation of TB is necessary. OBJECTIVE: To assess the efficacy of PCR for TB detection and to correlate with granuloma histology and AFB staining. METHODS: We analyzed 380 fixed paraffin-embedded tissues (PETs) of granulomas with and without caseous necrosis; suppurative; sarcoidal; or of chronic nonspecific nature. Nested PCR-IS6110 for Mycobacterium tuberculosis complex (MTB) and a nested pan-Mycobacterium for the hsp65 gene were used for Mycobacterium spp detection. RESULTS: PCR was more sensitive than AFB staining for all five catagories of granulomas: G1: PCR 71%, AFB staining 28%. G2: PCR 37%, AFB 8%. G3: PCR 17%, AFB staining 7%. G4: PCR 8%, AFB staining 4%. G5: PCR 6%, AFB staining 0%. CONCLUSIONS: Molecular diagnosis of TB using PCR-based testing is a fast, efficacious and sensitive method that increased the accuracy of PET histological diagnosis associated with granulomatous lesions.

La citometría de flujo aumenta la proporción de muestras valorables en líquido cefalorraquídeo con recuento celular normal en hemopatías malignas

Introducción: El compromiso del líquido cefalorraquídeo (LCR) en hemopatías malignas es un marcador de mal pronóstico y es habitualmente estudiado por citometría de flujo o citología. Ocasionalmente, las muestras de LCR oligocelulares (≤ 5 céls/dL) pueden ser consideradas como no aptas para diagnóstico por la baja cantidad de eventos. Objetivo: Evaluar la proporción de muestras reportadas como valorables para diagnóstico obtenidas por citometría y citología en muestras de LCR oligocelular. Material y Métodos: Se seleccionaron 169 muestras de LCR oligocelular correspondientes a 115 pacientes con hemopatías malignas. Las muestras fueron obtenidas mediante punción lumbar en tubos acondicionados con EDTA y preservante celular (Transfix®). El inmunofenotipo se realizó con panel de 8 colores, 55 (32%) de las cuales se hizo con panel para pequeñas muestras (SST). En todos los casos se incluyó CD14 para identificación de monocitos y CD3 para linfocitos T. La adquisición se realizó en citómetro FACSCantoII® y el análisis en software Infinicyt®. Resultados: La proporción de muestras valorables fue mayor en citometría en comparación con la citología (98% vs 61%, p < 0,000). En la mayoría se identificaron linfocitos T (98%) y/o monocitos (90%). En las muestras con SST, la cantidad de eventos obtenida fue menor en muestras con < de 1 mL (140 vs 556, p < 0,001) y se logró identificar una mediana de 3 poblaciones celulares. Conclusión: La citometría proporciona una mayor cantidad de muestras valorables en los LCR paucicelulares en relación con la citología en muestras de LCR enviadas para estudio de compromiso de LCR por hemopatías malignas.

Background: The alteration of cerebrospinal fluid (CSF) in hematologic neoplasms is a poor prognostic marker. The characteristics of CSF are usually analyzed by flow cytometry or cytology. However, paucicellular CSF samples (≤5 cells/dL) can sometimes be considered unsuitable for analysis due to the low number of events. Objective: To evaluate the proportion of samples reported as suitable for analysis obtained by cytometry (FCM) and cytology in paucicellular CSF samples. Material and Methods: 169 samples ofpaucicellular CSF corresponding to 115 patients with hematologic neoplasms were selected. The samples were obtained by lumbar puncture in tubes conditioned with EDTA and Transfix®. We characterized the immunophenotype ofCSF samples with an 8-color panel, and 55 samples (32%) were in a small sample tube (SST). In all cases, monocytes were identified by CD14 labeling and T lymphocytes by CD3 labeling. The acquisition was carried out in a FACSCantoII® cytometer, and the analysis was performed using Infinicyt® software. Results: The proportion of samples suitable for analysis was higher in FCM compared to cytology (98% vs 61%, p < 0.000). We identified the presence of T lymphocytes and/or monocytes in most samples (98% and 90%, respectively). In the SST samples, the number of events recorded in low-volume samples (< 1 mL) was lower than in samples with higher volume (140 vs 556, p < 0.001), with a median of identification of 3 cell populations. Conclusion: FCM allows the analysis of a higher proportion ofpaucicellular CSF samples than cytology in hematologic neoplasms study.

Paraganglioma Cardíaco: reporte de un caso / Cardiac Paraganglioma: case report

Abstract: A 49-year-old woman presented with dyspnea and palpitations, leading to Functional Class III.An echocardiogram showed a heterogeneous mass adhered to the right heart cavities. This was confirmed by NMR. A large right coronary artery was occluded in relation to the tumor, which was hyper vascularized. Resection of the tumor was performed; the right ventricular wall was sutured, and an atrial defect was closed using pericardial tissue. Post operative course was uneventful and she was asymptomatic 4 years after surgery.

Orbito-ethmoidal rhabdomyosarcoma in an adult patient: a case report and review of the literature.

We report a patient who presented to the ENT service complaining of nasal obstruction, exophthalmos, edema and ipsilateral facial congestion. Imaging studies revealed an aggressive noncalcified solid mass centered in the left nasoethmoidal region and heterogeneous avid enhancement following contrast media injection. Subsequently, a biopsy confirmed the presence of solid alveolar rhabdomyosarcoma. The patient was treated with chemoradiation therapy for 7 weeks. Due to the advanced stage of the disease, the patient was enrolled in a palliative care and pain control program.

Mieloma múltiple en pacientes con lupus eritematoso sistémico: presentación de dos casos / Multiple myeloma in systemic lupus erythematosus: report of two cases

There is an increased incidence of hematological malignancies, particularly non-Hodgkin lymphoma (NHL) in systemic lupus erythematosus (SLE). In contrast, the concurrence with Multiple Myeloma (MM) is very rare, and the possible pathogenetic mechanisms underlying this association remain unclear. We report two patients who developed MM 15 and four years after being diagnosed as having SLE.

Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central: caso clínico / Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: report of one case

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 year-old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.

Eficacia del diagnóstico diferencial de tuberculosis mediante reacción de polimerasa en cadena en lesiones granulomatosas de tejidos fijados en formalina e incluidos en parafina / Efficacy of PCR for the differential diagnosis of tuberculosis in granulomatous lesions of paraffin-embedded formalin fixed tissues

Background: Granulomatous lesions occur in tuberculosis (TB), other infections, toxic, allergic, and autoimmune diseases among others. In absence of a an acid-fast bacilli (AFB) confirmation of TB is necessary. Objective: To assess the efficacy of PCR for TB detection and to correlate with granuloma histology and AFB staining. Methods: We analyzed 380 fixed paraffin-embedded tissues (PETs) of granulomas with and without caseous necrosis; suppurative; sarcoidal; or of chronic nonspecific nature. Nested PCR-IS6110 for Mycobacterium tuberculosis complex (MTB) and a nested pan-Mycobacterium for the hsp65 gene were used for Mycobacterium spp detection. Results: PCR was more sensitive than AFB staining for all five catageories of granulomas: G1: PCR 71%, AFB staining 28%. G2: PCR 37%, AFB 8%. G3: PCR 17%, AFB staining 7%. G4: PCR 8%, AFB staining 4%. G5: PCR 6%, AFB staining 0%. Conclusions: Molecular diagnosis of TB using PCR-based testing is a fast, efficacious and sensitive method that increased the accuracy of PET histological diagnosis associated with granulomatous lesions.

Introducción: Lesiones granulomatosas ocurren en tuberculosis (TBC), otras infecciones, condiciones tóxicas, alérgicas y autoinmunes, entre otras. Con baciloscopia negativa, es necesario confirmar el diagnóstico de TBC. Objetivo: Evaluar la eficacia de la RPC para detectar TBC comparado con baciloscopia en relación a la histología del granuloma. Métodos: Analisis de 380 tejidos fijados en formalina e incluidos en parafina (TFFP) con diferentes tipos de granulomas: con necrosis caseosa; sin necrosis caseosa; supurativo; sarcoidal; a cuerpo extraño/inespecífico. Utilizamos RPC anidada-IS6110 para detección del complejo Mycobacterium tuberculosis (MTB) y una pan-RPC anidada-hsp65 para Mycobacterium spp. Resultados: La detección de TBC mediante RPC fue significativamente superior a baciloscopia en los cinco tipos de granuloma: G1: RPC 71%, baciloscopia 28%; G2: RPC 37%, baciloscopia 8%; G3: RPC 17%, baciloscopia 7%; G4: RPC 8%, baciloscopia 4%; G5: RPC 6%, baciloscopia 0%. Conclusión: El diagnóstico de TBC por RPC es un método rápido, eficaz y de gran sensibilidad, que aumenta la precisión del diagnóstico diferencial de lesiones granulomatosas de TFFP procesados rutinariamente en histopatología.

Diagnóstico diferencial de tuberculosis en base a PCR en lesiones granulomatosas clasificadas histopatológicamente / PCR-based differential diagnosis of tuberculosis in histopathologically classified granulomatous lesions

El diagnóstico diferencial de tuberculosis en tejidos fijados en formalina e incluidos en parafina es necesario porque la morfología de la lesión tuberculosa es variada, hay diversos granulomas clasificados en necrobióticos, tuberculoideos, supurativos, sarcoideo, a cuerpo extraño/crónico inespecífico. Las lesiones granulomatosas ocurren en tuberculosis y también en otras infecciones (hongos, parásitos, brucelosis, lepra) en condiciones tóxicas, alérgicas, autoinmunes, tumores y otras. El diagnóstico histológico no es confirmatorio de tuberculosis y en ausencia de una baciloscopia positiva, se hace necesaria la confirmación molecular para el diagnóstico diferencial. Evaluamos la eficacia de la técnica de PCR para la detección de tuberculosis en tejidos fijados y comparamos esos resultados con la histología del granuloma y la baciloscopia. Analizamos 444 biopsias de diferentes tejidos (ganglios, piel, pleura, pulmón, intestino, tejido óseo, mama y otros) de 5 tipos de granulomas: G1.tuberculoideo con necrosis caseosa; G2.tuberculoideo sin necrosis caseosa; G3. supurativo; G4. sarcoideo l; G5. a cuerpo extraño/inespecífico. Utilizamos dos PCR-IS6110 nested para detección del complejo Mycobacterium tuberculosis y un pan PCR-hsp65 nested para detección de Mycobacterium spp. Los resultados obtenidos muestran que la detección de tuberculosis mediante PCR fue significativamente superior que mediante baciloscopia. G1: PCR 69,6%, baciloscopia 31,3%; G2: PCR 26,8%, baciloscopia 6,1%; G3: PCR 16,7%, baciloscopia 6,7%; G4: PCR 7%, baciloscopia 4%; G5: PCR 6,7%, baciloscopia 0%. Concluimos que el diagnóstico molecular de tuberculosis mediante un PCR robusto adaptado a tejidos fijados es eficaz, rápido, sensible y contribuye a la precisión del diagnóstico diferencial en diferentes tipos de granulomas (AU)

The differential diagnosis of tuberculosis in fixed paraffin embedded-tissues is necessary due to both the diverse morphology of tuberculous lesions and the varying histological types of granulomas (necrobiotic, tuberculoid, suppurative, sarcoidal and foreign body/inespecific). Granulomatous lesions occur in tuberculosis, in other infections (fungal, parasitic, brucelosis, lepra), in toxic, allergic and autoimmune, tumours and in conditions of unknown etiology. Diagnosis of tuberculosis cannot be confirmed by histopathology alone and in absence of a positive acid-fast bacilli (AFB) stain, molecular confirmation of tuberculosis is necessary for a correct differential diagnosis. The aim of our study was to assess PCR efficacy for mycobacterial infection detection in fixed tissues and to correlate those findings with granuloma histology and with AFB staining. We analyzed 444 biopsies from various tissues (lymph nodes, skin, pleura, lung, intestine, bone tissue, breast and others) with 5 granuloma types: G1: with caseous necrosis; G2: without caseous necrosis; G3: suppurative; G4: sarcoidal; G5: chronic/nonspecific. For molecular detection, we used nested PCR-IS6110 for Mycobacterium tuberculosis complex and a nested pan PCR-hsp65 for Mycobacterium sp.. The results obtained demonstrated that PCR was significantly better than AFB stain for tuberculosis detection. G1: PCR 69.6%, AFB staining 31.3%. G2: PCR 26.8%, AFB staining 6.1%; G3: PCR 16.7%, AFB staining 6.7%; G4: PCR 7%, AFB staining 4%. G5: PCR 6.7%, AFB staining 0%. We conclude that molecular diagnosis of tuberculosis using robust PCR-based testing adapted to fixed tissues is a fast, efficient and sensitive method that increases the accuracy of the differential diagnosis of granulomatous lesions (AU)

Tumor neuroblástico en el niño / Neuroblastic tumor in children

Los tumores neuroblásticos son tumores del sistema nervioso periférico. Algunos inmaduros como el neuroblastoma tienen comportamiento muy agresivo, mientras otros como el ganglioneuroma son considerados benignos. Por su variada sintomatología, al neuroblastoma se le denomina "el gran simulador" y es caracterizado como enigmático. El dolor es la principal manifestación, así como también la distensión abdominal y la palpación de una masa abdominal.