Artrite reumatoide do tipo EORA com trombocitose significativa: relato de caso / Elderly-onset rheumatoid arthritis with significant thrombocytosis: a case report

Este relato teve como objetivo apresentar um caso de elderly onset rheumatoid arthritis associada à trombocitose reacional significativa. À admissão, o paciente apresentava quadro de poliartrite de pequenas e grandes articulações associado à rigidez matinal. Após exames solicitados, evidenciaram-se trombocitose de 1.697.000 cel./mm³ e anticorpos antipeptídeos citrulinados positivos, sendo diagnosticado com artrite reumatoide do tipo elderly onset rheumatoid arthritis.

This report aimed at presenting a case of elderly-onset rheumatoid arthritis associated with significant reactive thrombocytosis. On admission, the patient presented polyarthritis of small and large joints associated with morning stiffness. After the performance of the requested tests, thrombocytosis of 1,697,000 cells/mm3 and positive anti-CCP were evidenced, and the patient was diagnosed with elderly-onset rheumatoid arthritis.

Diagnostic value of the neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and thrombocytosis in the preoperative investigation of ovarian masses / Valor diagnóstico das razões neutrófilo/linfócito, plaqueta/ linfócito, e trombocitose na investigação pré-operatória de massas ovarianas

Abstract Objective To evaluate the diagnostic accuracy of cancer antigen 125 (CA125) and complete blood count (CBC) parameters, such as the neutrophil to lymphocyte ratio (NLR), the platelet to lymphocyte ratio (PLR), and thrombocytosis in patients with ovarian masses. Methods The present is a retrospective study conducted at a single tertiary hospital from January 2010 to November 2016. We included consecutive women referred due to suspicious adnexal masses. The CBC and CA125 were measured in the serum of 528 women with ovarian masses before surgery or biopsy. We evaluated the diagnostic performance of the NLR, PLR, platelets (PLTs), CA125, and the associations between them. We tested the clinical utility of the CBC parameters and CA125 in the discrimination of ovarian masses through decision curve analysis (DCA). Results The best balance between sensitivity and specificity was obtained by the associations of CA125 or PLTs ≥ 350/nL, with 70.14% and 71.66%, CA125 or PLTs ≥ 400/ nL, with 67.30% and 81.79%, CA125 or PLR, with 76.3% and 64.87%, and CA125 or NLR, with 71.09% and 73.89% respectively. In the DCA, no isolated CBC parameter presented a higher clinical utility than CA125 alone. Conclusion We showed that no CBC parameter was superior to CA125 in the prediction of the malignancy of ovarian tumors in the preoperative scenario.

Resumo Objetivo Avaliar a acurácia diagnóstica do antígeno de câncer 125 (cancer antigen 125, CA125, em inglês) e dos parâmetros do hemograma como as razões neutrófilo/linfócito (RNL), plaqueta/linfócito (RPL), e trombocitose em pacientes com massas ovarianas. Métodos Este é um estudo retrospectivo realizado em um hospital terciário no período de janeiro de 2010 a novembro de 2016. Foram incluídas de forma consecutiva mulheres encaminhadas por massas anexiais suspeitas. Foram dosados hemogramas e CA125 no soro de 528 mulheres com massas ovarianas antes da cirurgia ou biópsia. Foram avaliados os desempenhos diagnósticos da RNL, da RPL, das plaquetas (PLQs) e do CA125, considerando-os isoladamente e associados entre si. Testamos a utilidade clínica dos parâmetros do hemograma e do CA125 na discriminação das massas ovarianas por análise de curva de decisão (ACD). Resultados Os melhores equilíbrios entre sensibilidade e especificidade foram obtidos por meio das associações do CA125 ou PLQs ≥ 350/nL, com 70,14% e 71,66%, CA125 ou PLQs ≥ 400/nL, com 67,30% e 81,79%, CA125 ou RPL, com76,3% e 64,87%, e CA125 ou RNL, com 71,09% e 73,89%, respectivamente. Conclusão Na ACD, nenhum parâmetro do hemograma isolado se mostrou superior ao CA125 na predição de malignidade de tumores ovarianos no pré-operatório.

Características citomorfológicas de las alteraciones plaquetarias cuantitativas y su relación con otras alteraciones celulares / Cytomorphological characteristics of quantitative platelet alterations and its association with other cell alterations

Introducción: Las alteraciones cuantitativas de plaquetas son producidas por el incremento o disminución de los conteos globales de plaquetas. El incremento o trombocitosis se produce por redistribución o aumento de la producción medular; la disminución puede ser el resultado de una reducción de la producción, redistribución o acortamiento de la sobrevida de las plaquetas en circulación. Objetivo: Describir los hallazgos citomorfológicos más importantes en las alteraciones cuantitativas de plaquetas. Métodos: Se realizó una revisión de la literatura, en inglés y español, en la base de datos PubMed y el motor de búsqueda Google Académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la bibliografía revisada. Análisis y síntesis de la información: Las alteraciones cuantitativas de plaquetas se caracterizan por variaciones en el número y morfología de estas células. Estas se asocian a causas congénitas o adquiridas, en la que la detallada anamnesis de los pacientes es un elemento importante en el diagnóstico. En la trombocitosis se debe diferenciar una trombocitosis reactiva de una enfermedad medular primaria; mientras que en la trombocitopenia se debe considerar el origen étnico de los pacientes y la morfología de los leucocitos. Son numerosas las causas hereditarias de trombocitopenia con anomalías morfológicas de plaquetas y granulocitos. Conclusiones: Las alteraciones cuantitativas de plaquetas son un amplio número de entidades con semejanzas y diferencias en cuanto a presentación y manifestaciones clínicas. Los exámenes de laboratorio constituyen una herramienta importante en el diagnóstico, pronóstico y el seguimiento de los pacientes afectados(AU)

Introduction: Quantitative platelet alterations are produced by the increase or decrease in global platelet counts. Platelet count increase or thrombocytosis is produced by redistribution or increased marrow production. Platelet decrease may result from production, redistribution, or shortened survival of circulating platelets. Objective: To describe the most significant cytomorphological findings in quantitative platelet alterations. Methods: A literature review was carried out, in English and in Spanish, in the database PubMed and with the search engine of Google Scholar, of articles published in the last ten years. An analysis and summary of the revised bibliography was made. Information analysis and synthesis: Quantitative platelet alterations are characterized by variations in the number and morphology of these cells. These are associated with congenital or acquired causes, in which detailed anamnesis of patients is an important element in the diagnosis. In thrombocytosis, reactive thrombocytosis must be differentiated from primary marrow disease; while in thrombocytopenia, the ethnic origin of the patients and the morphology of the leukocytes must be considered. Hereditary causes of thrombocytopenia with morphological abnormalities of platelets and granulocytes are numerous. Conclusions: Quantitative platelet alterations are a large number of entities with similarities and differences in terms of presentation and clinical manifestations. Laboratory tests are an important tool for diagnosis, prognosis, and follow-up of affected patients(AU)

A subgroup analysis of JUMP, a phase IIIb, expanded-access study evaluating the safety and efficacy of ruxolitinib in patients with myelofibrosis in a Brazilian cohort

Abstract Introduction Ruxolitinib has been approved for the treatment of myelofibrosis (MF). In this study, we present safety and efficacy findings from an analysis of 104 patients with intermediate- and high-risk MF in a Brazilian cohort of the JUMP study who received treatment with ruxolitinib. Methods JUMP is a single-arm, open-label, phase IIIb, expanded-access study. The primary endpoint was to evaluate the safety and tolerability (frequency, duration, and severity of adverse events [AEs]) of ruxolitinib. Results All of the 104 patients received the treatment. Median duration of exposure was 35.8 months. The most common hematologic AEs were anemia (57.7), thrombocytopenia (38.5%), neutropenia (11.5%), and leukopenia (9.6%). Second malignancies (all grades) occurred in 19.2% of patients (n = 20). Serious AEs were reported in 62.5% of patients (n = 65). The proportions of patients with ≥50% reduction from baseline in palpable spleen length at weeks 24 and 48 were 62.7% and 69.2%, respectively. The mean change from the baseline in the Functional Assessment of Cancer Therapy (FACT)-Lymphoma total score was 10.8 [15.6%] at week 4, 12.6 [14.1%] at week 24, and 12.2 [14.3%] at week 48. The mean change from the baseline for the Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue scale was 3.9 [42.8%] at week 4, 4.9 [29.9%] at week 24, and 4.7 [28%] at week 48. At week 48, the estimated progression-free survival, leukemia-free survival, and overall survival probabilities were 91%, 91% and 93%, respectively Overall, 21 deaths were observed in the present study. Conclusion Findings from this study suggest that ruxolitinib could be evaluated as a standard-of-care treatment for the MF population in need of a viable treatment option. NCT01493414

Trombose essencial: uma revisão da literatura / Essential thrombosis: a literature review

Trombose essencial é uma das doenças mieloproliferativas crônicas, rara e de etiologia ainda desconhecida, mas que apresenta risco alto de eventos trombóticos e/ou hemorrágicos, uma vez que acomete as células megacariocíticas e, consequentemente, as plaquetas. O objetivo deste trabalho foi realizar uma revisão das publicações sobre o tema abordado. O estudo caracteriza-se como revisão bibliográfica de artigos das bases de dados da Literatura Latino-Americana e do Caribe em Ciências da Saúde (Lilacs), National Library of Medicine (PubMed), Scientific Electronic Library Online (SciELO) e Google acadêmico. Foram incluídos artigos disponíveis e em qualquer idioma de publicação, com a finalidade de aumentar o referencial teórico. Posteriormente à seleção e análise dos artigos, foram encontrados alguns pontos em comum, como a dificuldade em se diagnosticar a doença. De acordo com os estudos, a maioria dos doentes é assintomática, mas pode apresentar desde eventos trombóticos até mesmo embolia pulmonar. Atualmente, o tema vem crescendo, principalmente abordando técnicas moleculares mais específicas para a descoberta da doença em seu estágio inicial. A análise dos artigos demonstrou a dificuldade do diagnóstico da trombose essencial, sendo a sua identificação crucial nos estágios iniciais.

Essential thrombosis is one of the chronic myeloproliferative diseases, rare and of unknown etiology, but which presents a high risk of thrombotic and / or hemorrhagic events. Once it attacks the megakaryocytic cells and consequently the platelets. The objective of this work is to review the publications on the subject. The study is characterized as a bibliographical review of articles from the Latin American and Caribbean Literature in Health Sciences (LILACS), National Library of Medicine (PubMed), Scientific Electronic Library Online (SciELO) and Google Acadêmico. Articles were included and in any language of publication, in order to increase the theoretical reference. Subsequent to the selection and analysis of the articles, some common points were found such as the difficulty in diagnosing the disease. According to the studies, the majority of patients are asymptomatic but may present from thrombotic events to even pulmonary embolism. Currently, the topic has been growing, mainly addressing molecular techniques more specific to the discovery of the disease in its initial stage. The analysis of the articles demonstrated the difficulty of diagnosing essential thrombosis, which is crucial in the initial stages.

Doença celíaca: revisão bibliográfica e relato de caso / Celiac disease: literature review and case report

RESUMO A doença celíaca (DC) é uma forma crônica de enteropatia de mecanismo imunológico que afeta o intestino delgado de crianças e adultos geneticamente predispostos, precipitada pela ingestão de alimentos contendo glúten. Também é conhecida como espru celíaco, enteropatia sensível ao glúten ou espru não tropical. A doença pode se manifestar na forma Clássica, Não Classica e assintomática. Para o diagnostico é imprescindível a realização de endoscopia digestiva alta com biópsia de intestino delgado, considerado o padrão-ouro. Os marcadores sorológicos são úteis para identificar os indivíduos que deverão ser submetidos à biópsia de intestino delgado e também são úteis para acompanhamento do paciente celíaco. Os principais testes sorológicos para a detecção da intolerância ao glúten são o anticorpo antigliadina, o anticorpo antiendomísio e o anticorpo antitransglutaminase (TTG). O tratamento da DC consiste na introdução de dieta isenta de glúten de forma permanente, devendo-se, portanto, excluir da dieta os seguintes cereais e seus derivados: trigo, centeio, cevada, malte, aveia. Neste trabalho relatamos o caso de um paciente com diagnóstico tardio de DC, que iniciou o quadro com enteropatia perdedora de proteína, com quadro infeccioso vigente, PCR aumentado, leucocitose, plaquetose e hipoalbuminemia. Realizada EDA com biópsia que juntamente com anti endomisio IgA positivo resultaram no diagnóstico de DC. Paciente manteve-se com leucocitose e plaquetose. O objetivo deste relato é apresentar uma paciente com diagnóstico de DC e revisar aspectos clínicos e terapêuticos atuais da doença. Palavras-chave: Doença Celíaca; Enteropatia Perdedora de Proteína ; Plaquetose; Leucocitose.

Prevalencia, características demográficas y causas de trombocitosis y trombocitopenia en neonatos ingresados al servicio de neonatología del Hospital Nacional de Niños Benjamín Bloom durante el año 2016 / Prevalence, demographic characteristics and causes of thrombocytosis and thrombocytopenia in neonates admitted to the neonatology service of the Benjamin Bloom National Children's Hospital during 2016

Los neonatos son susceptibles a diversas enfermedades en los que juega un rol importante la detección temprana. Con el uso del conteo electrónico de plaquetas como parte del hemograma de rutina, la trombocitosis y trombocitopenia se observan con mayor frecuencia como un hallazgo inesperado, y en nuestro medio no se encuentran descripciones respecto al tema, por lo que se realizará el siguiente estudio. Se realizó un estudio descriptivo, transversal, retrospectivo. De una población de 589 ingresados, se seleccionaron los casos con conteos plaquetarios mayores de 450,000 plaquetas o menores de 150,000 plaquetas. Se excluyeron 18 pacientes que presentaban criterios de exclusión, y 6 pacientes cuyos expedientes no fueron encontrados. Quedando 76 sujetos sometidos al estudio. La edad media de la población que presentó alteración cuantitativa de plaquetas fue de 8 días. Con sexo masculino de 61%. La prevalencia trombocitosis fue del 5% y la de trombocitopenia del 7%. Para todos los grupos de edad gestacional se obtuvo una mayor frecuencia de trombocitopenia. La mayor parte de los casos encontrados corresponden a alteraciones leves. Se observó trombocitopenia con una frecuencia tres veces mayor que la de trombocitosis en los neonatos en las categorías de bajo peso. En la población con sepsis y sospecha de sepsis se encontró una mayor frecuencia de trombocitosis. Únicamente 8% de la población neonatal con trombocitosis o trombocitopenia presentaba anemia

Isomerismo derecho (síndrome de ivemark): presentación de un caso / Right isomerism (ivemark syndrome): presentation of a case

El Síndrome de Ivemark se clasifica como un trastorno de Heterotaxia o un trastorno de lateralidad de baja incidencia, que debuta con manifestaciones clínicas caracterizados por latidos cardiacos a la derecha y asplenia entre otras malformaciones, se puede asociar a un patrón de herencia autosómico recesivo que es el más frecuente. Se destaca que entre las malformaciones más frecuentes del aparato digestivo se encuentra la mal rotación intestinal la cual fue descartada y los signos de distensión abdominal y mala tolerancia alimentaria se debió a la descompensación gradual del paciente. El diagnóstico prenatal mediante la ecocardiografía es pieza clave en el manejo posterior del paciente, debido que permite programar su nacimiento en centros de III nivel, por su alta complejidad y requerimiento de cirugía cardiotorácica, el manejo terapéutico de asplenia determina el pronóstico del paciente.

The Ivemark Syndrome is classified as a Heterotaxia disorder or a low incidence laterality disorder, which debuts with the clinical characteristics for right heartbeats and asplenia among other malformations, it can be associated with an autosomal recessive inheritance pattern that is the most frequent It is emphasized that the most frequent, malformations of the digestive system are in the phase of abdominal rotation that the discarded and the signs of abdominal distension and food intolerance were due to the gradual decompensation of the patient. Prenatal diagnosis through echocardiography is the key to the management of the birth of the patient, which allows to schedule its birth in the centers of III level, for its high complexity and requirement of cardiothoracic surgery, the therapeutic management of Asplenia determines the prognosis of the patient.

Factores de riesgo asociados a complicaciones por Bordetella pertussis en la Fundación Hospital de la Misericordia, 2009-2013 / Risk factors associated with complications of Bordetella pertussis at the Fundacion Hospital de la Misericordia, 2009-2013

Objetivo: Caracterizar factores de riesgo asociados a complicaciones en los pacientes con tosferina en 2009-2013. Materiales y métodos: Estudio de casos y controles. Identificación de factores de riesgo asociados a complicaciones en pacientes con Bordetella pertussis , confirmados con reacción en cadena de la polimerasa. Resultados: Se caracterizó a 96 pacientes. Un total de 76% eran menores de 6 meses, media de 4,56 ± 6,8 meses. El 58,3% eran niños y el 41,5%, niñas. Un 51% tuvo alguna complicación. Al 58% se les hospitalizó en cuidado crítico. Un 9,4% tuvo sepsis y el 14,6% requirió ventilación asistida. Un 4,2% fallecieron. La media de estancia en cuidado crítico fue de 8,75 días ( ± 85) y hospitalaria de 13,92 días ( ± 12,81). Se identificaron los siguientes factores de riesgo asociados a complicación: noción de contagio (OR: 2,20), ausencia de vacunación (OR: 3,31). Clínica: cianosis (OR: 4,07), deshidratación (OR: 2,72), taquicardia (OR: 2,64), taquipnea (OR: 3,94), desaturación (OR: 3,34). Paraclínica: coinfección bacteriana (OR: 7,66), linfocitosis (OR: 3,18), trombocitosis (OR: 3,038). Conclusión: Los antecedentes, clínica y paraclínicos pueden definir pacientes con infección por Bordetella pertussis en riesgo de complicaciones e ingreso en cuidado crítico.

Objective: To characterize the risk factors associated with complications in patients infected with Bordetella pertussis , 2009-2013. Material and methods: Case-control study. Identification of risk factors associated with complications in patients with Bordetella pertussis , confirmed by polymerase chain reaction. Results: A total of 96 patients were characterized. 76% were younger than 6 months, mean 4.56 ± 6.8 months; there were 58.3% boys and 41.5% girls. A total of 51% had complications. Some 58% were hospitalized in critical care. Sepsis occurred in 9.4% patients, and 14.6% required assisted ventilation. A total of 4.2% died. The mean critical care stay was 8.75 days ( ± 85) and the mean hospital stay was 13.92 ± 12.81 days. The following risk factors associated with complications were identified: notion of contagion (OR: 2.20), no vaccination (OR: 3.31). Clinical: (OR: 4.07) cyanosis, dehydration (OR: 2.72), tachycardia (OR: 2.64), tachypnea (OR: 3.94), desaturation (OR: 3.34). Paraclinical: Bacterial co-infection (OR: 7.66), lymphocytosis (OR: 3.18), thrombocytosis (OR: 3.038). Conclusion: Background, clinical and paraclinical factors can identify patients with B. pertussis infection at risk for complications and admission to the critical care unit.

Pseudohiperkalemia por trombocitosis esencial: una entidad pobremente reconocida / Pseudohyperkalaemia in essential thrombocytosis: a poorly recognized

INTRODUCCIÓN: La hiperkalemia representa una emergencia médica frecuentemente manejada en servicios de urgencia. Identificar una pseudohiperkalemia en pacientes que presentan recuentos plaquetarios elevados permite evitar terapias innecesarias e iatrogenia que pueden tener consecuencias potencialmente fatales. PRESENTACIÓN DEL CASO: Mujer de 73 años, con enfermedad renal crónica (ERC) etapa-3a (etiología no precisada). En consulta neurológica por crisis isquémica transitoria (abril 2011), se demostró hiperkalemia de 6,0 mEq/L (suero), tasa de filtración glomerular(TFG) de 51 mL/min y trombocitosis de 1.113.000 plaquetas/mm3. Se trata la hiperkalemia con furosemida y dieta pobre en potasio. Inició hidroxiurea indicada por hematología. En control nefrológico (diciembre 2012) presenta hiperkalemia de 7,5 mEq/L (suero), TFG de 37 ml/min y trombocitosis de 1.052.000 plaquetas/mm3. Electrocardiograma (ECG), sin signos de cardiotoxicidad acordes al nivel de kalemia. Se sospechó pseudohiperkalemia secundaria a trombocitosis esencial (PSTE), se suspendió atenolol, furosemida y se continuó hidroxiurea. En febrero de 2013 presentó kalemia de 6,1 mEq/L (suero), trombocitosis de 713.000 plaquetas/mm3 y TFG de 31 mL/min. En mayo de 2014 la kalemia en suero fue de 5,5 mEq/L y en plasma de 5,2 mEq/L; trombocitosis de 503.000plaquetas/mm3 y TFG de 23 ml/min, confirmándose la PSTE. DISCUSIÓN: Hiperkalemia asociada a trombocitosis exige descartar PSTE. La regresión de la kalemia junto con la trombocitosis y ECG sin alteraciones en hiperkalemia, apoyan este diagnóstico. Su confirmación exige demostrar una hiperpotasemia mayor en suero que en plasma (0,36 +/-0,18 meq/L). Tratar una pseudohiperkalemia, puede generar iatrogenia (hipokalemia y/o hemoconcentración que aumenta riesgo de trombosis). Se descarta hiperkalemia secundaria a ERC con TFG>15ml/min

INTRODUCTION: Hyperkalemia is a medical emergency often managed in emergency services. Identifying a pseudohiperkalemia in patients with high platelet counts will avoid unnecessary iatrogenic therapies, which can have potentially fatal consequences. CASE REPORT: 73 year old woman, with chronic kidney disease (CKD) stage-3a (unknown etiology). In neurological consultation by transient ischemic attack (april 2011) it was evidenced a hyperkalemia of 6.0 mEq/L (serum), glomerular filtration rate (GFR) of 51 mL/min and thrombocytosis of 1,113,000 platelets/mm3. Hyperkalemia is treated with furosemide and potassium poor diet. Started on hydroxyurea indicated by hematology. Nephrology consultant (december 2012) realized hyperkalemia of 7.5 mEq/L (serum), GFR of 37mL/min and thrombocytosis of 1,052,000 platelets/mm3. EKG with no sign of cardiotoxicity was found in relation to potassium serum level. A pseudohyperkalemia secondary to essential thrombocytosis (PSET) was suspected. Atenolol and furosemide were discontinued and hidroxiurea was continued. In February 2013 potassium serum level was 6.1 mEq/L, platelet count: 713,000/ mm3 and GFR: 31 mL/min. In may 2014 serum potassium was 5.5 mEq/L and plasma potassium was 5.2 mEq/L; platelet count: 503,000//mm3 and GFR: 23 mL/min, confirming PSET. DISCUSSION: Hiperkalemia associated with thrombocytosis requires to rule out PSET. Regression of serum potassium with platelet count and the abscense of EKG changes with simultaneous hyperkalemia, support this diagnosis. Confirmation of PSTE diagnosis needs to demonstrate a potassium serum level greater than in plasma (0.36 +/- 0.18 mEq/L). Treating a pseudohiperkalemia can generate iatrogenia (hypokalemia and/or hemoconcentration, increasing the risk of thrombosis). A hyperkalemia secondary to CKD is ruled out with GFR >15mL/min

Evaluation of oral-motor movements and facial mimic in patients with head and neck burns by a public service in Brazil

OBJECTIVES: The purpose of this study was to analyze the characteristics of oral-motor movements and facial mimic in patients with head and neck burns. METHODS: An observational descriptive cross-sectional study was conducted with patients who suffered burns to the head and neck and who were referred to the Division of Orofacial Myology of a public hospital for assessment and rehabilitation. Only patients presenting deep partial-thickness and full-thickness burns to areas of the face and neck were included in the study. Patients underwent clinical assessment that involved an oral-motor evaluation, mandibular range of movement assessment, and facial mimic assessment. Patients were divided into two groups: G1 - patients with deep partial-thickness burns; G2 - patients with full-thickness burns. RESULTS: Our final study sample comprised 40 patients: G1 with 19 individuals and G2 with 21 individuals. The overall scores obtained in the clinical assessment of oral-motor organs indicated that patients with both second- and third-degree burns presented deficits related to posture, position and mobility of the oral-motor organs. Considering facial mimic, groups significantly differed when performing voluntary facial movements. Patients also presented limited maximal incisor opening. Deficits were greater for individuals in G2 in all assessments. CONCLUSION: Patients with head and neck burns present significant deficits related to posture, position and mobility of the oral myofunctional structures, including facial movements. .

Síndrome POEMS: a propósito de un caso y revisión de la literatura / POEMS syndrome: report of a case and review of literature

El síndrome POEMS es un síndrome paraneoplásico raro asociado a discrasia de células plasmáticas. Se presenta el caso de un paciente masculino de 37 años de edad, quien consultó por presentar de 38.5 C°, precedida de escalofríos, predominio nocturno y frecuencia diaria/interdiaria, de tres meses de evolución; concomitante parestesias en región plantar bilateral, ascendente, con disminución de fuerza muscular simétrica en miembros inferiores. Durante su estancia hospitalaria se evidencia trombocitosis y eritrocitosis. Se realizó ecosonograma abdominal que evidencia hepatomegalia; tomografía computarizada (TC) de cuello que reportó adenopatías submandibulares, laterocervicales y parayugulares; TC de tórax donde se evidencia lesión ocupante de espacio de características osteolíticas expansivas a nivel de segunda costilla izquierda; electromiografía de miembros superiores e inferiores con radiculopolineuropatía de tipo mixto en miembros inferiores y polineuropatía sensitivo motora de tipo mixto en miembros superiores; electroforesis de proteínas con discreto aumento de gammaglobulinas, a expensas de IgG y aspirado de médula ósea con serie plasmática aumentada (3%) de apariencia madura. Se realizó biopsia de lesión en segundo arco costal izquierdo sin evidenciar atipias. En vista de hallazgos clínicos y paraclínicos se planteó síndrome POEMS.

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. We present a male patient of 37 years old with fever 38.5°C, preceded by chills, nocturnal predominance and daily frequency of three months of evolution, as well as paresthesias in plantar region, bilateral, ascendant with decrease in muscle strength of legs. During his hospitalization, he presented thrombocytosis and erythrocytosis. The abdominal ecosonography evidenced hepatomegaly; thorax computerized tomography evidenced space osteolytic occupying lesion in second left rib. The electromyography demonstrated radiculo polineuropathy in legs, and sensory motor polineuropathy in upper limbs. The protein electrophoresis with elevated of gammaglobulins ;bone marrow aspirate evidenced (3%) mature increased plasma cells. The biopsy of second rib evidenced no atypia.

Queilitis granulomatosa como parte del síndrome de Melkersson Rosenthal / Granulomatous cheilitis as a part of Melkersson Rosenthal syndrome

La queilitis granulomatosa es una entidad infrecuente caracterizada por tumefacción labial recurrente. Es considerada la forma incompleta más frecuente del síndrome de Melkersson Rosenthal, compuesto por la tríada de edema orofacial recurrente, lenguafisurada y parálisis facial. En su histología es característica la presencia de granulomas no caseificantes. Presentamos el caso de una paciente con queilitis granulomatosa y revisamosla bibliografía. Se destaca además esta entidad por su difícil manejo y variables respuestas a los tratamientos instaurados...

Trombocitosis esencial: comunicación de dos casos / Essential thormbocytosis: report of two cases

La trombocitosis esencial (TE) es una panmielopatía clonal de escasa frecuencia, que está incluida dentro de los síndromes mieloproliferativos crónicos. Se caracteriza por la proliferación anormal de los megacariocitos, y las manifestaciones clínicas principales son: hemorragia y/o diátesis tromboembólica, en cuyo caso se presentan fenómenos necróticos y ulcerosos. Se presentan dos pacientes de sexo masculino de 54 y 80 años, cuyas primeras manifestaciones fueron lesiones úlcero-necróticas cutáneas. Con recuentos plaquetarios mayores a 1.000.000 mm3 y la punción biopsia de médula ósea que reveló hiperplasia megacariocítica, se arriba al diagnóstico de TE.

Essential thrombocytosis (ET) is a rare, clonal panmyelopathy and one of a group of disordersknown as chronic myeloproliferative diseases.ET is associated with sustained megakaryocyte proliferation, and clinically presents hemorrhage orthrombosis with ulcerative or necrotic lesions.We report two male patients aged 54 and 80-years-old, respectively, with ulcerative and necroticlesions on the skin as the first manifestation of this disease. The platelet count was higher than1.000.000 mm3 and the bone marrow aspirate biopsy showed megakaryocytic hyperplasiadiagnostic of ET.

Trombocitose como fator prognóstico no câncer colorretal / Trombocitosis as a prognostic factor in colorectal cancer

Objetivos - Investigar o significado prognóstico da trombocitose nos pacientes com câncer colorretal. Método - Trata-se de estudo retrospectivo, com análise de 243 prontuários de pacientes submetidos a operações por câncer colorretal. Foram comparados os dados do estadiamento, recidiva tumoral e óbitos por câncer com a ocorrência de trombocitose no pré-operatório. O grupo controle foi composto de 50 pacientes submetidos à herniorrafia. A média da contagem de plaquetas no pré-operatório destes pacientes foi utilizada para dividir os pacientes em dois grupos: grupo 1, pacientes com contagem de plaquetas abaixo dessa média e grupo 2, pacientes com contagem de plaquetas acima dessa média. Resultados - A média da contagem plaquetária foi 317000/ìl entre os pacientes com câncer e de 267000 entre os pacientes do grupo controle. A prevalência da trombocitose no câncer colorretal foi 32,1 por cento. Dentre os óbitos por câncer, 56,7 por cento ocorreram em pacientes com trombocitose e 32 por cento em pacientes com plaquetas normais (p=0,001). Utilizando a média do grupo controle, a diferença foi ainda mais significativa (p=0,0004). Quanto à recidiva tumoral, 40 por cento dos pacientes do grupo 2 tiveram recidiva e 17,9 por cento do grupo 1 (p=0,003). Com relação ao estadiamento T, no grupo 1, 14,1 por cento eram T1 e 8,4 por cento T4. No grupo 2, 2,2 por cento eram T1 e 19,5 por cento T4 (p=0.0005). Metástases à distância foram encontradas em 9,4 por cento dos pacientes do grupo 1 contra 21,8 por cento do grupo 2 (p=0.02). No que diz respeito ao estadiamento TNM, no grupo 1, 24,6 por cento eram estadio 1 e 11 por cento estadio 4. No grupo 2, 9,6 por cento eram estadio 1 e 22,8 por cento estadio 4 (p=0,02). Conclusão - A contagem de plaquetas no pré-operatório parece ser útil em identificar pacientes com prognóstico desfavorável.

Aims - To investigate the prognostic value of thrombocytosis in patients with colorectal cancer. Method - Charts of 243 patients with colorectal cancer that had undergone surgical treatment were retrospectively reviewed. Data on TNM staging, tumor recurrence and cancer death were compared with the occurrence of thombocytosis before surgery. The control group was formed by fifty patients submitted to herniorrhaphy. Patients were divided into two groups on the basis of mean platelet count of control group: group 1, patients with platelet count below that mean and group 2, patients with platelet count above that level. Results - The mean platelet count was 317000/ìl among cancer patients and 267000/ìl in the control group patients. Prevalence of thrombocytosis in patients with colorectal cancer was 32,1 percent. The cancer death rate was 56,7 percent in patients with thrombocytosis and 32 percent in patients with normal platelet count (p=0,001). When the platelet count mean of control group was used, the difference was significantly greater (p=0004). Tumor recurrence occurred in 40 percent of group 2 patients and in 17,9 percent of group 1 patients (p=0,003). Regarding T staging, on group 1, 14,1 percent of patients were T1 and 8,4 percent were T4. On group 2, 2,2 percent were T1 and 19,5 percent were T4 (p=0,0005). Distant metastasis were found in 9,4 percent patients of group 1 and 21,8 percent of group 2 (p=0,02). Considering TNM staging, group 1 had 24,6 percent of patients in stage 1 and 11 percent in stage 4. On group 2, 9,6 percent belonged to stage 1 and 22,8 percent to stage 4 (p=0,02). Conclusion - Preoperative platelet count seems to be a helpful indicator of patients with poor prognosis.