Síndrome de la persona rígida asociado a timoma / Stiff person syndrome associated with thymoma

Resumen El síndrome de persona rígida es un cuadro neuro lógico infrecuente caracterizado por rigidez muscular de tronco y extremidades y espasmos musculares ga tillados por estímulos sensoriales o emocionales, que progresa hacia la postración. Cuenta con un mecanismo fisiopatogénico con base inmunológica, en el cual los autoanticuerpos, como el antiGAD65, cumplen un rol central. Asimismo, la detección de dichos anticuerpos corrobora el diagnóstico ante un paciente con cuadro clínico sugestivo. Un 4 a 6% de los casos tienen neoplasias subyacentes. El tratamiento se basa en el manejo sintomático, inmunomodulador y de la enfermedad de base en los casos paraneoplásicos. Reportamos un caso de síndrome de persona rígida clásico asociado a timoma y describimos las características principales de esta entidad.

Abstract Stiff-person syndrome is a rare neurological condi tion characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostra tion. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detec tion of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We re port a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

Ictericia no obstructiva como síndrome paraneoplásico del carcinoma de próstata: revisión sistemática de casos publicados / Non-obstructive jaundice as paraneoplastic syndrome of prostate carcinoma: Systematic review of published cases

El síndrome de Stauffer es un síndrome paraneoplásico (SPN) en el que se produce una afectación hepática; clásicamente se ha relacionado con tumores renales, pero también con otros tumores como el adenocarcinoma de próstata (ACP). Nuestro objetivo es llevar a cabo una revisión sistemática de los casos publicados asociados al cáncer de próstata. El número de artículos cribados en la revisión bibliográfica fue de 357, de los cuales 25 cumplieron los criterios de inclusión. Todos los casos publicados de síndrome de Stauffer en pacientes con ACP estaban en estadio metastásico. El SPN se resolvió en 3 de cada 4 pacientes cuando se instauró el tratamiento dirigido al cáncer de próstata. La aparición del SPN en pacientes ya diagnosticados de ACP, niveles no elevados de bilirrubina total y la no resolución del SPN se presentaron como factores de mal pronóstico. (AU)

Stauffer syndrome is a paraneoplastic syndrome (PS) that involves liver disorders; it has been often related to renal tumors, but also to others such as adenocarcinoma of the prostate (ACP). Our objective was to carry out a systematic review of published cases associated with ACP. A total of 357 articles were accessed, 25 of which met the study's inclusion criteria. All published cases of Stauffer syndrome in patients diagnoses with ACP were in the metastatic stage. The PS resolved in 3 out of 4 patients when ACP-targeted therapy was implemented. The following were identified as poor prognosis factors: the diagnosis of ACP prior to that of SP, non-elevated levels of total bilirubin, and the non-resolution of SP at the start of treatment. (AU)

[Non-obstructive jaundice as paraneoplastic syndrome of prostate carcinoma: Systematic review of published cases]. / Ictericia no obstructiva como síndrome paraneoplásico del carcinoma de próstata: revisión sistemática de casos publicados.

Stauffer syndrome is a paraneoplastic syndrome (PS) that involves liver disorders; it has been often related to renal tumors, but also to others such as adenocarcinoma of the prostate (ACP). Our objective was to carry out a systematic review of published cases associated with ACP. A total of 357 articles were accessed, 25 of which met the study's inclusion criteria. All published cases of Stauffer syndrome in patients diagnoses with ACP were in the metastatic stage. The PS resolved in 3 out of 4 patients when ACP-targeted therapy was implemented. The following were identified as poor prognosis factors: the diagnosis of ACP prior to that of SP, non-elevated levels of total bilirubin, and the non-resolution of SP at the start of treatment.

Retinopatía autoinmune asociada a la presencia de anticuerpos onconeuronales: a propósito de un caso / Autoimmune retinopathy with onconeuronal antibodies: Case report

Se presenta el caso de retinopatía autoinmune en un paciente con carcinoma microcítico de pulmón, no conocido hasta el momento, que se diagnosticó tras la exploración oftalmológica. La serología fue positiva para anticuerpos onconeuronales CV2/CRMP5. La retinopatía autoinmune es una entidad rara que puede pasarse por alto, y ser infradiagnosticada. Se produce por una reacción inmunomediada contra antígenos retinianos. La importancia de su diagnóstico precoz radica en que en muchos de los pacientes la sintomatología ocular aparece antes del diagnóstico del cáncer primario, por lo que su identificación y derivación precoz para estudio de extensión puede suponer el diagnóstico de una neoplasia primaria oculta hasta el momento. (AU)

We present a case of autoimmune retinopathy in a patient with unknown small cell lung cáncer (SCLC), which was diagnosed after ophthalmological examination. Serology was positive for CV2/CRMP5 onconeuronal antibodies. Autoimmune retinopathy is a rare entity that can be missed and underdiagnosed. It is produced by an immune-mediated reaction against retinal antigens. The importance of its early diagnosis lies in the fact that in many of the patients, ocular symptoms appear before the diagnosis of the primary cancer, so its early identification and referral for an extension study may lead to the diagnosis of a hidden primary neoplasm. (AU)

Autoimmune retinopathy with onconeuronal antibodies: Case report.

We present a case of autoimmune retinopathy in a patient with unknown small cell lung cáncer (SCLC), which was diagnosed after ophthalmological examination. Serology was positive for CV2/CRMP5 onconeuronal antibodies. Autoimmune retinopathy is a rare entity that can be missed and underdiagnosed. It is produced by an immune-mediated reaction against retinal antigens. The importance of its early diagnosis lies in the fact that in many of the patients, ocular symptoms appear before the diagnosis of the primary cancer, so its early identification and referral for an extension study may lead to the diagnosis of a hidden primary neoplasm.

Síndrome paraneoplásico de Doege-Potter: manejo paliativo / Doege-Potter syndrome: palliative care

El síndrome de Doege-Potter es un síndrome paraneoplásico excepcional asociado a los tumores fibrosos solitarios, otra entidad muy poco común. Se caracteriza por hipoglucemias sintomáticas mantenidas por la sobreproducción de factor de crecimiento insulínico tipo 2, una molécula muy similar a la insulina. La intervención quirúrgica es la única opción para evitar la aparición de este síndrome. En formas inoperables, la quimioterapia ha demostrado eficacia en el control de la hipoglucemia. No obstante, el uso de corticoides es la opción terapéutica indicada en el control de síntomas derivados de esta entidad cuando el paciente no es candidato a ningún otro tratamiento agresivo, mejorando su calidad de vida. (AU)

Doege-Potter syndrome is a rare paraneoplastic syndrome associated with solitary fibrous tumors, which also represent another uncommon disease. It is characterized by symptomatic hypoglyce- mia due to overproduction of insulin growth factor type 2, a molecule similar to insulin. Surgical treatment is the only option to prevent this syndrome from developing. In inoperable cases, chemotherapy has shown efficacy in the management of hypoglycemia. Nevertheless, the use of corticosteroids has been recognized as the best therapeutic option to keep symptoms under control when the patient is not a candidate to any other agressive treatment, improving quality of life. (AU)

Cáncer de mama triple negativo asociado a dermatomiositis paraneoplásica: Reporte de caso y revisión de la literatura / Triple-negative breast cancer associated with paraneoplastic dermatomyositis: Case report and literature review

Resumen La dermatomiositis (DM) es un tipo de miopatía inflamatoria bien definida, inmunomediada, con afectación específica del músculo esquelético y con compromiso variable de piel y otros órganos. Se caracteriza por debilidad muscular proximal, lesiones cutáneas patognomónicas de dermatomiositis como el signo de Gottron, eritema violáceo o heliotropo, y evidencia de inflamación muscular por enzimas elevadas, cambios miopáticos en electromiografía y biopsia muscular anormal. Tiene una asociación bien establecida con diferentes tipos de cáncer pero es rara su asociación con cáncer de mama. Cuando se presentan de manera concomitante, su diagnóstico requiere un estudio multidisciplinario para orientar el origen paraneoplásico frente a una etiología propiamente autoinmune que requiera terapia inmunosupresora dirigida. Describimos el caso de una paciente con diagnóstico simultáneo de carcinoma infiltrante de mama triple negativo y criterios de dermatomiositis como manifestación paraneoplásica.

Abstract Dermatomyositis (DM) is a well-defined immune-mediated inflammatory myopathy, with specific involvement of skeletal muscle and variable involvement of skin and other organs. It is characterized by proximal muscle weakness, pathognomonic skin lesions of dermatomyositis such as Gottron's sign, violaceous or heliotrope rash, and evidence of muscle inflammation due to elevated enzymes, myopathic changes on electromyography, and abnormal muscle biopsy. It has a well-established association with different types of cancer, but its association with breast cancer is rare. When they occur concomitantly, their diagnosis requires a multidisciplinary study to confirm the paraneoplastic origin versus a primarily autoimmune etiology that may require targeted immunosuppressive therapy. We describe the case of a patient with a simultaneous diagnosis of triple-negative infiltrating breast carcinoma and criteria for dermatomyositis as a paraneoplastic manifestation.

Síndrome miasteniforme de Lambert-Eaton / Lambert-Eaton myasthenic syndrome

Introducción: El síndrome miasteniforme de Lambert-Eaton (LEMS) es una patología paraneoplásica (T-LEMS) o idiopática autoinmunitaria (NT-LEMS) ocasionada por autoanticuerpos contra los canales de calcio dependientes del voltaje presinápticos de la unión neuromuscular. El 60% de los T-LEMS se asocia a carcinoma de pulmón de células pequeñas. Una puntuación Dutch-English LEMS Tumor Association Prediction (DELTA-P) mayor de 3 denota un riesgo elevado de dicha asociación. El diagnóstico precoz fundado en los hallazgos clínicos, estudios neurofisiológicos y dosificación de títulos de anticuerpos en el suero permite iniciar tempranamente el tratamiento sintomático y la búsqueda oncológica. Son escasos los informes de pacientes con LEMS en Latinoamérica. Objetivo: Describir las características de pacientes con LEMS de un centro privado de Buenos Aires, Argentina, y compararlas con las de otras series publicadas. Pacientes y métodos: Se revisaron historias clínicas de 13 pacientes con LEMS con hallazgos clínicos, electromiograma compatible y/o anticuerpos positivos. Se realizó seguimiento hasta descartar o confirmar una neoplasia asociada de acuerdo con los algoritmos recomendados. Resultados: Cuatro pacientes presentaron diagnóstico de T-LEMS, dos de ellos con carcinoma de pulmón de células pequeñas. De los nueve pacientes con NT-LEMS, cinco presentaron una puntuación DELTA-P de 3 y 4. Nueve pacientes presentaron la tríada clínica clásica desde el inicio. Todos los pacientes presentaron en el electromiograma hallazgos compatibles con defecto de placa neuromuscular presináptico. El 70% mejoró sintomáticamente con piridostigmina. Conclusiones: Los hallazgos clínicos, junto con los estudios neurofisiológicos compatibles, resultan suficientes para el diagnóstico de LEMS. No pudo replicarse la relación entre puntuación DELTA-P y riesgo de carcinoma de pulmón de células pequeñas...(AU)

Introduction: Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological screening. Reports of patients with LEMS in Latin America are scarce. Aim: This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. Patients and methods: The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. Results: Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. Conclusions: The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.(AU)

Dermatomiositis y cáncer colorrectal: reporte de caso y revisión de la literatura / Dermatomyositis and colorectal cancer: Case report and literature review

Resumen Los pacientes con dermatomiositis presentan un mayor riesgo de asociación con patología maligna subyacente, sin conocerse una causa precisa de esta relación. Son múltiples las posibles localizaciones anatómicas, entre las que se incluyen los ovarios, mamas, zona gástrica, zona colorrectal, sangre, pulmones y próstata. Se presenta el caso de una mujer de 58 años que durante el estudio de pérdida anormal de peso manifestó debilidad muscular grave y alteraciones dermatológicas asociadas con el hallazgo de adenocarcinoma del colon transverso.

Abstract Patients with dermatomyositis are more likely to have an underlying malignancy, although the exact cause of this association is unknown. There are multiple possible anatomical sites, including ovaries, breasts, stomach, colorectum, blood, lungs, and prostate. We present the case of a 58-year-old woman who during abnormal weight loss study showed severe muscle weakness and skin alterations, associated with finding of adenocarcinoma of the transverse colon.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Policondritis recidivante como fenómeno paraneoplásico en paciente con síndrome mielodisplásico / Relapsing Polychondritis as paraneoplastic phenomenon in patient with Myelodysplastic Syndrome

Los síndromes mielodisplásicos son un grupo heterogéneo de enfermedades hematológicas, caracterizadas por hematopoyesis ineficaz con riesgo de progresión a leucemia mieloide aguda. Pueden asociarse a manifestaciones autoinmunes en un 10-30% de los pacientes, apareciendo antes, durante o luego del diagnóstico del trastorno hematológico. La prevalencia de policondritis recidivante como fenómeno paraneoplásico es de 0,7-5,4%, presentándose de forma simultánea en la mayoría de los casos. Otros procesos autoinmunes asociados incluyen: vasculitis sistémica, poliartritis seronegativa, dermatosis neutrofílica, citopenias inmunomediadas, presencia de autoanticuerpos y crioglobulinemia. Reportamos el caso de una mujer de 60 años, sin antecedentes patológicos previos, que presentó un cuadro de policondritis recidivante y vasculitis sistémica asociadas a síndrome mielodisplásico.

Myelodysplastic syndromes are a heterogeneous group of hematological diseases, characterized by ineffective hematopoiesis with risk of progression to acute myeloid leukemia. They can be associated to autoimmune manifestations in 10-30% of patients, appearing before, during or after the diagnosis of the hematological disorder. The prevalence of relapsing polychondritis as a paraneoplastic phenomenon is 0.7-5.4%, occurring simultaneously in the majority of cases. Other associated autoimmune processes include: systemic vasculitis, seronegative polyarthritis, neutrophilic dermatosis, immunomediated cytopenias, presence of autoantibodies and cryoglobulinemia. We report the case of a 60-year-old woman, with no previous medical history, who presented with recurrent polychondritis and systemic vasculitis associated with myelodysplasia.

[Multiple insufficiency fractures (metatarsial, calcaneal and sacral) as the first manifestation of lung adenocarcinoma. A case report and literature review]. / Fracturas múltiples por insuficiencia (metatarsianos, calcáneo y sacro) como signo inicial de adenocarcinoma pulmonar. Caso clínico y revisión de la literatura.

Insufficiency fractures, or pathological fractures, are produced after minimal trauma or no prior trauma and normally affect weakened bone. Their presence should be suspected in fractures showing abnormal patterns, when several fractures occur in a short period of time and in those with no apparent or only minimal trauma. On confirmation of an insufficiency fracture, a differential diagnosis should be made between tumoral and metastatic fractures if there is a history of underlying primary malignancy. The epidemiology of lung cancer has changed due to women's adoption of smoking. In women, the most frequent type of lung cancer is adenocarcinoma, which is less aggressive and has lower mortality, with 5-year survival of 9.5%. Consequently, in Spain, 44% of pulmonary nodules are due to adenocarcinomas. Therefore, all solitary pulmonary nodules should be followed-up for at least 5 years. A history of solitary pulmonary nodule is found in one out of every 1,000 x-rays. However, in patients younger than 35 years, only 1% is malignant. In persons with a history of malignancy and age older than 35 years, this percentage increases to 68%. Size larger than 3cm increases the percentage of malignancy to up to 93%. Therefore, in female smokers older than 50 years with a history of solitary pulmonary nodule, the nodule should be considered malignant until demonstrated otherwise. We report for the first time in Spain the case of a woman with risk factors (smoking, age older than 50 years, with a 3-cm solitary pulmonary nodule that showed no significant growth in 3 years) who had multiple insufficiency fractures in a short period of time. Rehabilitation therapy was unsuccessful and the patient underwent investigation for malignancy. Unfortunately, histopathological study confirmed that the fractures were the initial manifestation of lung adenocarcinoma.

Lymphoma and unilateral diffuse uveal melanocytic proliferation: when both conditions co-exist in the eye. / Linfoma y proliferación melanocítica uveal difusa unilateral: cuando ambas entidades coexisten en el ojo.

The case is presented of a 60 year-old male with decreased visual acuity in his left eye (LE). In the funduscopic examination, pigmentary alterations associated with sub-retinal orange plaques could be observed that, in autofluorescence, manifested as hypoautofluorescent spots. Subsequently, the patient subsequently developed significant vitritis with large white sub-retinal plaques on the posterior pole of his LE, with no alterations in the right eye. The IL-10/IL-6 coefficient was 0.87, and a lesion suggestive of a lymphoma in the frontal lobe could be seen on brain magnetic resonance scan. The patient was diagnosed with primary lymphoma of the central nervous system with ocular involvement of the LE and associated unilateral diffuse uveal melanocytic proliferation of the same eye. Diffuse uveal melanocytic proliferation is a bilateral para-neoplastic process that occurs as a consequence of carcinomatous tumour processes, although it does not always meet these characteristics.

Síndrome de Stauffer como manifestación de carcinoma renal de células claras / Stauffer's syndrome as manifestation of clear cell renal carcinoma

Resumen: La incidencia de carcinoma renal de células claras representa cerca de 2% de todas las neoplasias malignas. La tríada clásica de hematuria, dolor abdominal y masa palpable se estima en 10% de los casos. Stauffer describió en 1961 el primer caso llamado síndrome de disfunción hepato-nefrogénica no metastásica, este padecimiento puede encontrarse en 3 a 20% de los casos. La afectación de las enzimas hepáticas puede estar en relación con la infiltración metastásica del hígado o, bien, por disfunción no metastásica. Es una manifestación poco frecuente en cáncer renal. Ante el hallazgo de enzimas hepáticas y fosfatasa alcalina elevadas y al descartar alteración hepática anatómica y funcional, debe sospecharse el síndrome de Stauffer, lo que puede hacer la diferencia entre que éste pase inadvertido o establecer el diagnóstico de manera oportuna. Se comunica una manifestación poco frecuente de síndrome paraneoplásico en cáncer renal de células claras.

Abstract: The incidence of clear cell renal carcinoma corresponds to about 2% of all malignant neoplasms. The classic triad of hematuria, abdominal pain and palpable mass is estimated in 10% of cases. Stauffer described in 1961 the first case called nonmetastatic hepatophrenic dysfunction syndrome, this condition can be found in 3 to 20% of cases. The involvement of liver enzymes may be related to metastatic liver infiltration or also due to non-metastatic dysfunction. It is a rare manifestation in renal cancer. Before the finding of elevated liver enzymes and alkaline phosphatase, ruling out both anatomic and functional hepatic impairment, Stauffer syndrome should be suspected, being able to make the difference between going unnoticed or being a timely finding. This paper reports a little frequent manifestation of paraneoplastic syndrome of clear cell renal carcinoma.

Mesotelioma pleural con manifestaciones paraneoplásicas de osteoartropatía hipertrófica / Pleural mesothelioma with paraneoplastic manifestations of hypertrophic osteoarthropathy

RESUMEN Se presenta un paciente exfumador de 65 años que, un año antes de comenzar las manifestaciones respiratorias, comenzó con dolores óseos erráticos, tratados con medicamentos comunes hasta que es atendido por disnea, tos y dolor torácico en el Hospital Básico San Antonio, ciudad de Riobamba, provincia de Chimborazo, Ecuador. El paciente presenta un gran derrame pleural, del cual obtienen 1500 ml de líquido serohemático, cuyo estudio citológico es positivo de malignidad. Se somete a cirugía, se confirma histológicamente mesotelioma maligno en etapa IV y se trata posteriormente con quimioterapia, pero el paciente empeora progresivamente hasta fallecer. Los autores resaltan que las manifestaciones paraneoplásicas del cáncer del pulmón son más frecuentes cuando la localización es parenquimatosa y no pleural, y llaman la atención sobre el hecho de que en este paciente comenzaron 1 año antes de que aparecieran los síntomas respiratorios.

ABSTRACT We present a 65-year-old ex-smoker patient, in which respiratory manifestations with erratic bone pain treated with common medications, began a year before he was atended for dyspnea, cough and chest pain at "San Antonio" Basic Hospital, Riobamba city, Chimborazo province, Ecuador. The patient presented a large pleural effusion of 1500 ml of serohematic fluid. Cytological study was positive for malignancy to rule out mesothelioma. Surgery was performed, histologically malignant mesothelioma was confirmed in stage IV and treated with chemotherapy. But the patient worsens progressively until death. Authors emphasized that lung cancer paraneoplastic manifestations were more frequent in not pleural and parenchymal location. They also called attention to patients´ symptoms, which began one year before the respiratory condition appeared.

Síndrome de Sweet asociado a Leucemia Mieloide Aguda: Reporte de un caso en Perú / Sweet Syndrome associated with Acute Myeloid Leukemia: Case report in Peru

Se presenta el caso de paciente mujer de 79 años que presenta fiebre, placas eritemato-violáceas y ampollas de contenido seroso, dolorosas en el antebrazo y dorso de la mano izquierda.Los hemogramas seriados revelaron la presencia de anemia, leucocitosis marcada con neutrofilia, trombocitopenia y linfopenia. Se realiza lámina periférica donde se observa blastos (79%) y cuerpos de Auer. La biopsia de la lesión cutánea señala un infiltrado denso perivascular de neutrófilos maduros en la dermis superficial sin vasculitis asociada. Se diagnosticó Síndrome de Sweet asociado a Leucemia mieloide aguda, iniciando terapia de acuerdo a protocolo.El síndrome de Sweet es una afección cutánea poco frecuente de causa desconocida que se caracteriza por aparición brusca de fiebre, afectación del estado general y lesiones tipo placas o nódulos eritematosos muy dolorosos, con o sin pseudovesículas, de predominio en el hemicuerpo superior. Además, es frecuente leucocitosis, neutrofilia y PCR elevado. (AU)

We present the case of a 79-year-old female patient with fever, erythematous-violaceous plaques and blisters of serous content, painful on the forearm and back of the left hand.Serial blood tests revealed the presence of anemia, leukocytosis marked with neutrophilia, thrombocytopenia and lymphopenia. Peripheral lamina is where blastos (79%) and Auer bodies are observed. The biopsy of the skin lesion indicates a dense perivascular infiltrate of mature neutrophils in the superficial dermis without associated vasculitis. Sweet syndrome associated with acute myeloid leukemia was diagnosed, initiating therapy according to protocol.Sweet syndrome is a rare skin condition of unknown cause that is characterized by sudden onset of fever, general condition impairment and very painful plaque or erythematous nodule lesions, with or without pseudovesicles, predominantly in the upper hemibody. Also, leukocytosis, neutrophilia and elevated CRP are frequent. (AU)

Glomeruloid hemangioma and POEMS syndrome. / Hemangiomas glomeruloides y síndrome POEMS.

POEMS syndrome is a paraneoplastic manifestation associated with hematopoietic disorders such as multiple myeloma and Castleman disease. POEMS is an acronym for the main clinical features of the syndrome, namely, Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin abnormalities. Glomeruloid hemangiomas are considered to be a specific clinical marker of POEMS syndrome. However, while they are not pathognomonic, their presence should raise suspicion of this syndrome or alert clinicians to its possible future development, as these lesions can appear years before the onset of the syndrome. We report the cases of 2 women with plasma cell dyscrasias and sudden onset of lesions with a vascular appearance and histologic findings consistent with glomeruloid hemangioma. Recognition of this vascular tumor is important for the early diagnosis of POEMS syndrome.

Síndromes paraneoplásicos en tumores gastrointestinales. Revisión de tema / A Review of Paraneoplastic Syndromes in Gastrointestinal Tumors

Resumen Los síndromes paraneoplásicos representan manifestaciones clínicas que producen los tumores en sitios distantes a ellos y que no están relacionadas físicamente con ellos ni con sus metástasis. Diferentes tumores gastrointestinales pueden presentar síndromes o manifestaciones sistémicas, dermatológicas, hematológicas, renales y neurológicas, entre otras. Aquí se ofrece una revisión de esas distintas manifestaciones.

Abstract Paraneoplastic syndromes produce tumors at sites distant from themselves and are not physically related to those tumors or to their metastases. Various gastrointestinal tumors may present syndromes or systemic, dermatological, hematological, renal, neurological and other manifestations. This study reviews these manifestations.

Polyarthritis and membranoproliferative glomerulonephritis as paraneoplastic manifestation of Hodgkin's lymphoma: A case report and literature review. / Poliartritis y glomerulonefritis membranoproliferativa como manifestaciones paraneoplásicas de linfoma de Hodgkin: presentación de un caso y revisión de literatura.

Paraneoplastic syndromes can be presented in multiple ways, which include endocrinological, hematologic, rheumatologic and nephrologic manifestations. While most of the publications described solid tumors as responsible for these manifestations, hematologic neoplasms are important cause to consider as part of the differential diagnosis. We report the case of a 46 year-old man with seronegative symmetric polyarthritis of large and small joints associated with membranoproliferative glomerulonephritis with deposits of immune complexes and acute impairment of renal function, as part of a paraneoplastic syndrome secondary of a classical Hodgkin lymphoma with bone marrow invasion, which reversed completely with chemotherapy treatment.

Dermatomiositis asociada a malignidad / Dermatomyositis associated with malignancy

La dermatomiositis es una enfermedad sistémica que se caracteriza fundamentalmente por la presencia de alteraciones inflamatorias de piel y músculo estriado. En ciertos casos constituye un síndrome paraneoplásico, por lo que su diagnóstico obliga a una exhaustiva búsqueda de la probable asociación con un cáncer. Presentamos tres casos de dermatomiositis asociados a neoplasias, dos mujeres con cáncer ginecológico y un varón con cáncer de estómago. Una de las mujeres también presentaba un síndrome mielodisplásico. En dos casos la dermatomiositis fue posterior al diagnóstico del cáncer y en uno los hallazgos fueron simultáneos.

Dermatomyositis is a systemic disease characterized primarily by the presence of inflammatory skin disorders and striated muscle. In some cases it constitutes a paraneoplastic syndrome, so diagnosis requires a thorough search of the likely association with cancer. We present three cases of dermatomyositis associated with malignancies, two women with gynecologic cancer and a man with stomach cancer. One of the women also had a myelodysplastic syndrome. In two cases dermatomyositis was after the diagnosis of cancer and in one, the findings were simultaneous.