Síndrome de hiperviscosidade em paciente com mieloma múltiplo / Hyperviscosity syndrome in multiple myeloma patients

O mieloma múltiplo é uma neoplasia progressiva e incurável de células B, caracterizado pela proliferação desregulada e clonal de plasmócitos na medula óssea. A síndrome de hiperviscosidade é uma das complicações relacionadas às gamopatias monoclonais, sendo considerada emergência oncológica. O objetivo deste estudo foi descrever o quadro clínico de um paciente diagnosticado com mieloma múltiplo que apresentou síndrome de hiperviscosidade, avaliando a prevalência de sinais e sintomas, bem como características fisiopatológicas dessa entidade clínica. Foi revisado o prontuário de um paciente internado na enfermaria da Clínica Médica do Hospital Regional do Cariri (CE) no período de junho a julho de 2018. Além disso, foi realizada revisão de literatura em base de dados (PubMed®) direcionada ao tema proposto. O diagnóstico de mieloma múltiplo foi comprovado por mielograma, sendo prontamente iniciada a corticoterapia e avaliada a resposta clínica após essa terapêutica. Apesar de incomum e menos frequentemente relacionada ao mieloma múltiplo, a síndrome de hiperviscosidade está relacionada a uma grande taxa de mortalidade quando apresenta diagnóstico tardio. A terapia de primeira linha indicada para a síndrome de hiperviscosidade foi a plasmaferese, no entanto, as condições clínicas (instabilidade hemodinâmica) impossibilitaram sua realização. O desfecho deste caso foi o óbito do paciente. Concluiu-se que o diagnóstico precoce e a intervenção terapêutica estão diretamente relacionados à ocorrência de menor incidência de complicações relacionadas ao mieloma múltiplo e à síndrome de hiperviscosidade.

Multiple myeloma is a progressive and incurable B-cell neoplasm characterized by unregulated and clonal proliferation of plasmocytes in the bone marrow. Hyperviscosity syndrome is one of the complications related to monoclonal gammopathies and is considered an oncological emergency. The aim of this study was to describe the clinical condition of a patient diagnosed with multiple myeloma who presented hyperviscosity syndrome, evaluating the prevalence of symptoms and signs, as well as the pathophysiological characteristics of this clinical entity. The medical records of a patient admitted to the Internal Medicine ward of the Hospital Regional do Cariri (CE) from June to July of 2018 were reviewed. In addition, we conducted a literature review in a database (PubMed®) directed to the theme proposed. The diagnosis of multiple myeloma was confirmed by myelogram, and corticosteroid therapy was promptly initiated and the clinical response was evaluated after this therapy. Although uncommon and less frequently related to multiple myeoloma, hyperviscosity syndrome is related to a high mortality rate when diagnosed late. The first line therapy indicated to hyperviscosity syndrome was plasmapheresis; however, the clinical conditions (hemodynamic instability) precluded its performance. The outcome of this case was the patient's death. Thus, it was concluded that early diagnosis and therapeutic intervention are directly related to the occurrence of lower incidence of complications related to multiple myeloma and hyperviscosity syndrome.

Carcinoma neuroendocrino de esófago: reporte de caso / Neoendocrine carcinoma of the esophagus: report of a case

El carcinoma neuroendocrino de esófago representa menos del 2% de casos de cáncer de esófago. Esta forma de presentación es extremadamente inusual, ya que este tipo de carcinoma se suele presentar con mayor frecuencia en zonas broncopulmonares. Los síntomas suelen asociarse a la obstrucción esofágica e incluyen odinofagia, disfagia y pérdida de peso, siendo infrecuente la clínica de síndrome carcinoide. Para el diagnóstico se requiere de uno o más de los siguientes marcadores de inmunohistoquímica positivos: cromogranina A, sinaptofisina o CD56 positivos, así como la presencia de los anticuerpos EMA y PANCK positivos. Se reporta el caso de un paciente varón con diagnóstico de tumor neuroendocrino de esófago.

Neuroendocrine carcinoma of esophagus represents less than 2% of cases of cancer of the esophagus. This presentation is extremely unusual, as this type of carcinoma typically presents with greater frequency in bronchopulmonary regions. The symptoms are usually associated with esophageal obstruction and include odynophagia, dysphagia and weight loss and are not usually associated with Carcinoid syndrome. The diagnosis requires one or more of the following positive immunohistochemical markers: Chromogranin A, synaptophysin or CD56 positive, as well as the presence of EMA and PANCK positive. We report the case of a male patient with diagnosis of neuroendocrine tumor of esophagus.

Coriocarcinoma gástrico primario: reporte de caso / Primary gastric choriocarcinoma: case report

El coriocarcinoma gástrico primario (CGP) es un tumor extremadamente raro, altamente invasivo y de rápida diseminación hematógena. Presentamos el caso de una paciente de 57 años que inicia con cuadro de hematemesis y, progresivamente, se le suman episodios de melena, baja de peso y epigastralgia. Es derivada al Instituto Nacional de Enfermedades Neoplásicas en donde se le realizan gastroscopía y biopsia. Así, el análisis histológico reportó patrón sugestivo para CGP; el cual se confirmó al realizarle a la paciente los estudios por imágenes necesarios y llevar a cabo el análisis inmunohistoquímico para gonadotrofina coriónica humana y alfa feto proteína. Posteriormente, a la paciente se le realiza una gastrectomía radical D2 con preservación esplénica y de cola de páncreas. Lamentablemente, su evolución no fue favorable y fallece por la progresión de la enfermedad.

Primary gastric choriocarcinoma (PGC) is an extremely rare and highly invasive tumor with rapid hematogenous spread. We present the case of a 57-year-old female patient who started with hematemesis and progressive episodes of melena, weight loss and epigastralgia. It is derived from the National Institute of Neoplastic Diseases where gastroscopy and biopsy are performed. Histological analysis reported pattern suggestive of PGC; that was confirmed by immunohistochemical analysis for human chorionic gonadotrophin and fetal alpha protein. Subsequently, the patient underwent a radical D2 gastrectomy with splenic preservation and tail of the pancreas preservation. Unfortunately, her evolution was not favorable and died due to the progression of the disease.

Clinical Efficacy of Dynamic Contrast-enhanced Multidetector-row Computed Tomography in Patients with Obscure Gastrointestinal Bleeding / 대한소화기학회지

BACKGROUND/AIMS: Obscure gastrointestinal bleeding (OGIB) accounts for 5% of all gastrointestinal (GI) bleeding cases. Dynamic contrast-enhanced multidetector-row CT (DCE-MDCT) is not generally recommended in OGIB patients due to its low sensitivity. However, it can be used to quickly and simply diagnose OGIB according to some guidelines. The aim of this study was to evaluate the clinical efficacy of DCE-MDCT in OGIB patients. METHODS: We retrospectively analyzed the medical records of 362 patients who underwent DCE-MDCT between March 2009 and January 2014. A total of 45 patients diagnosed with OGIB were included in this study. Their baseline characteristics and treatment procedure were analyzed retrospectively. The positive rates of DCE-MDCT for the detection of bleeding and associated factors were assessed. RESULTS: The mean age of the patients was 59 years, and males represented 51.1%. Melena was the most common symptom (44.4%). Positive rate of DCE-MDCT findings was 20.0% (9/45). Among these patients, intraluminal contrast extravasation was found in 5 patients (55.6%) and intraluminal hematoma or mass lesions were found in 2 patients each (22.2%). Thirty nine patients (86.7%) underwent conservative management, and 6 patients (13.3%) underwent specific treatment, such as endoscopic treatment, embolization, or surgery. Patients who showed positivity in DCE-MDCT more frequently received specific treatment compared with those who were negative (44.4% vs. 5.6%, p=0.010). CONCLUSIONS: Although DCE-MDCT showed a low positive rate (20.0%), positive findings of DCE-MDCT could lead to specific treatment. Positive DCE-MDCT findings play a useful role in the management of patients with OGIB.

Extramedullary relapse of multiple myeloma presenting as massive upper gastrointestinal bleeding: a rare complication

No abstract available.

Causes and outcome of upper gastrointestinal bleeding in emergency endoscopy unit of Ain Shams University Hospital

This prospective follow-up study was designed to analyze the causes and outcome of upper gastrointestinal bleeding among patients presenting by hematemesis and/or melena to Emergency Endoscopy Unit, Ain Shams University Hospitals. One thousand patients presented by upper GIT bleeding were subjected to complete clinical evaluation, emergency upper gastrointestinal endoscopy and therapeutic interventions as indicated. Follow up was done for occurrence of re-bleeding or mortality. Variceal causes of bleeding were the most common, representing 70.1% followed by non-variceal causes [26.1%] and obscure causes [3.8%]. Esophageal varices [EV] alone represented 17.8% of causes of variceal bleeding, while combined esophageal and gastric varices represented 39.5% and isolated gastric varices 12.8%. Gastric lesions were the most common causes of non variceal bleeding. Recurrence of bleeding occurred in 19.4% of variceal group in comparison to 6.1% of non variceal group, while mortality was found in 4.3% of variceal group in comparison to 1.5% of non variceal group with very highly significant difference [P <0.001]. Hypertension, ascites, EV columns, EV grade IV, presence of gastric varices and associated respiratory disorder were independent factors as-sociated with recurrence of bleeding in variceai group. In non variceal group, recurrence of bleeding was significantly related only to the presence of gastric ulcers [P=0.035]. Independent factors associated with mortality in studied patients were age, associated diabetes, presence of esophageal varices and associated duodenal ulcer

Upper gastrointestinal bleeding in children in southern Iran.

Objective. To find out whether the causes of upper GI bleeding in our center in a developing country differed from developed countries. Methods. Children presenting to our center with upper GI bleeding from March 2002 to March 2007, were retrospectively evaluated. Informations were retrieved from patient’s history and physical examination and results of upper GI endoscopy regarding etiology of bleeding, managements, use of medications which might predispose patient to bleeding, and the mortality rate. Results. From 118 children (67 boys; with age of 7.7±4.7 yrs) who underwent upper GI endoscopies, 50% presented with hematemesis, 14% had melena and 36% had both. The most common causes of upper GI bleeding among all patients were gastric erosions (28%), esophageal varices (16%), duodenal erosions (10%), gastric ulcer (8.5%), Mallory Weiss syndrome tear (7.8%), duodenal ulcer (6.8%), esophagitis (1.7%) and duodenal ulcer with gastric ulcer (0.8%). The causes of bleeding could not be ascertained in 20.5% of cases. No significant pre-medication or procedure related complications were observed. Endoscopic therapy was performed in 13.5% of patients. In 14.4% of patients, there was a history of consumption of medications predisposing them to upper GI bleeding. Two deaths occurred (1.7%) too. Conclusion. The findings in the present study showed that half of upper GI bleedings in pediatric patients from south of Iran, were due to gastric and duodenal erosions and ulcers. This study concludes that the causes of upper GI bleeding in children in our center of a developing country, are not different from those in developed ones.

Lesión de dieulafoy en colon: una causa inusual de hemorragia gastrointestinal en el paciente pediátrico / Colonic dieulafuy lesion: an unusual cause of gastrointestinal hemorrhage in children

La lesión de Dieulafoy es una causa importante pero infrecuente de hemorragia gastrointestinal. Se ha descrito a nivel gástrico y menos frecuente en duodeno, yeyuno y colon. Macroscópicamente es una malformación arterial, con tortuosidad y elongación del vaso, que aparece como un pequeño defecto de la mucosa e histológicamente son vasos largos que envuelven la submucosa y están por debajo de la mucosa. Tiene una incidencia del 0,3-1,5 por ciento como causa de sangrado gastrointestinal masivo. Pre-escolar masculino de cuatro años de edad quien ingresa por cuerpo extraño en vías aéreas (grano de maíz). Al estar hospitalizado presenta melenas con anemia e inestabilidad hemodinámica. Rectosigmoidoscopia con evidencia de sangrado descendente. Gammagrafía reporta zona de hipercaptación en ángulo esplénico de colon. En pabellón se realiza colonoscopia trans-operatoria, evidenciándose efusión sanguínea con puntillado hemorrágico en ángulo esplénico, por lo que se procede a realizar colectomía y anastomosis. Anatomía patológica reporta proliferación de vasos de mediano calibre en la submucosa, rodeados por estrona fibroso con infiltrado linfohistocitario, vasos congestivos en la serosa. Se plantea Lesión de Dieulafoy. Esta entidad clínica es infrecuente, el diagnóstico es difícil de realizar, cuando la ubicación es inusual. La gammagrafía es útil para el diagnóstico. Si el sangramiento digestivo compromete la vida del paciente el tratamiento definitivo es quirúrgico.

The Dieulafoy´s lesion is an infrequent but important cause of gastrointestinal haemorrhage. These lesions have been described at the gastric level and less frequently in the duodenum, jejune and colon. Macroscopically it is an arterial malformation, with tortuosity and elongation of the vessels that appear like a small defect in the mucosa and by histology as long vessels that surround the sub mucosa below the mucosa. It has an incidence of 0,3-1,5% as cause of massive gastrointestinal bleeding. 4 years old male that presents with a foreign body (corn seed) in the airway that required bronchoscophy. During admission presents frequent melena with anemia and hemodynamic instability. Rectosigmoidoscopy showed lower gastrointestinal bleeding without abnormal findings. Gammagram reported hypercaptant zone at the splenic angle of the colon. During surgery, trans-operating colonoscopy demonstrated hemorrhagic punctiform lesions with blood effusion at the splenic angle of the colon, and a segment of the colon was resected. Histopathology reported congestive blood vessels with proliferation of medium-caliber vessels, surrounded by fibrous stroma with linfohistiocitic infiltrate in the serosa, confirming the diagnose of Dieulafoy´s lesion. This is an infrequent cause of gastrointestinal bleeding that is difficult to diagnose when it presents in an inusual location. Gammagram is helpful for the diagnosis. If the gastrointestinal bleeding compromises the life of the patient, definite treatment should be surgical.

Hookworm infestation in children presenting with melena-case series

Hookworm infection is common in children and can present with symptoms of upper gastrointestinal bleeding and severe anemia. Ten children below 5 years presenting with melena and severe pallor were seen from December 2006 to May 2007 in the gastroenterology and hepatology department of children's hospital, Lahore. All patients had history of transfusion. Complete blood picture, eosinophil count with peripheral smear, stool complete examination for ova and cysts were performed in all cases, while upper and lower gastrointestinal endoscopies were performed in three patients to locate the source of bleeding. Stool routine examination in all these cases confirmed hook worm ova. These patients were managed with antihelmenthic and stool complete examination was done three days after the medicine. There was no mortality. Though upper gastrointestinal bleeding with hookworm infestation is very rare but in the developing countries it should be considered when other causes of upper gastrointestinal bleeding are ruled out

Clinical profile of dengue hemorrhagic fever cases in Mexico

OBJETIVO: El dengue hemorrágico en México es una enfermedad emergente desde 1994. La circulación de los cuatro serotipos incrementa el riesgo de epidemias de dengue hemorrágico. MATERIAL Y MÉTODOS: Se reportan los datos clínicos y epidemiológicos de los casos de dengue hemorrágico confirmados y notificados por el IMSS de 1995 a 2003. Se analizaron las características clínicas y epidemiológicas entre grupos. Para el control y la evaluación final de las variables se utilizó un modelo multivariado. RESULTADOS: Los casos fueron asignados en dos grupos: 438 con dengue clásico, que incluye 109 casos con manifestaciones hemorrágicas sin trombocitopenia, y 977 casos de dengue hemorrágico con 79 defunciones. Los factores de riesgo asociados a las defunciones fueron: hematemesis (RR 2.6; IC 95 por ciento 1.4-4.6) y melena (RR 2.2; IC 95 por ciento 1.2-3.7). CONCLUSIONES: El cuadro clínico descrito para la población del Instituto Mexicano del Seguro Social permite identificar factores pronósticos que ayuden al clínico a prevenir y manejar adecuadamente los casos severos de dengue hemorrágico.

Ectasia vascular antral gástrica: presentación clínica y manejo terapéutico / Gastric antral vascular ectasia: clinical presentation and therapeutic

La ectasia vascular antral gástrica (GAVE) o watermelon stomach es un causa de hemorragia digestiva alta cuyo reconocimiento se ha incrementado, la cual posee características endoscópicas e histológicas típicas. La enfermedad es más frecuente en mujeres ancianas y varias entidades asociadas han sido bien establecidas. Si bien algunos pacientes con gastropatía asociada a la hipertensión portal pueden presentar un aspecto similar a la GAVE, la distinción resulta de interés a los fines de elegir una terapéutica apropiada. El objetivo de este estudio es analizar las características clínicas y el tratamiento de un grupo de pacientes con GAVE evaluados en nuestra institución. Asimismo, se realizóuna revisión de la bibliografía actual. Seis mujeres tres hombres con GAVE fueron admitidos en el Hospital Británico de Buenos Aires entre noviembre de 1998 y enero de 2004. Se realizó biopsia endoscópica en ocho pacientes, siendo consistente con GAVE en todos los casos. Cuatro pacientes con anemia crónica como única manifestación fueron exitosamente tratados con suplementos de hierro. Se realizó tratamiento endoscópico en cuatro pacientes, tres de ellos con electrocauterio bipolar y uno con coagulación con argón plasma. El paciente restante fue sometido a antrectomía. Se observó resolución del sangrado en todos los casos, más allá de la terapéutica instituída.

The gastric antral vascular ectasia (GAVE) or watermelon stomach is an increasingly recognized cause of persistent upper gastrointestinal bleeding, which has typical endoscopic and histological findings. This disease is most frecuent in elderly women, and several associated conditions have been well established. Some patients with severe portal hypertensive gastropathy may have a GAVE like appearence. Nevertheless, a correct diagnosis is relevant in order to choose an appropiate treatment. The objective of the current study is to analize the clinical features and treatment of a group of patients with GAVE evaluated at our Institution. A review of the current literature was also performed. Six women and three men with GAVE were admitted at the Buenos Aires British Hospital between November 1998 and January 2004. Endoscopic biopsy was performed in eight patients and was consistent with GAVE in all cases. Four patients with chronic anaemia as unique manifestation were succesfully treated with iron supplements. Endoscopic treatment was performed in 4 patients, 3 of them were treated with bipolar electrocautery and 1 with argon plasma coagulation. An antrectomy was carried out in the ninth patient. The resolution of the bleeding was observed in all cases with the different therapeutics options used.

Idiopathic colonic varices.

Idiopathic varices of the colon are rare, with only a few reports in literature. We report 30-year-old man who presented with melena and had hepatic flexure varices detected at colonoscopy. Investigations for portal hypertension were negative. No treatment was given.

Pseudomelanosis duodeni: association with hypertension and chronic renal failure: case report.

We present the first reported case with typical endoscopic and histological findings from Thailand. An 80-year-old man presented with chronic periumbilical abdominal pain for 3 months and melena for one week. He had had hypertension for 17 years, chronic renal failure for 4 years and gouty arthritis for 3 years. Panendoscopy was done and showed diffusely scattered small black and brown pigmentation over the stomach and duodenum. Tissue biopsies from the black pigmented lesions were taken for further microscopic and histochemical evaluation. Histological finding and special histochemical stains, Fontana stain, revealed mild chronic inflammation with accumulation of hemosiderin pigment in the lamina propria of the stomach and duodenal villi. This condition is called Pseudomelanosis duodeni. The literature of this condition was also reviewed.

Gastric lipoma: a rare cause of gastrointestinal bleeding

We report the case of a 58 year old lady, admitted to hospital with gastrointestinal bleeding. Oesophago-gastroduodenoscopy demonstrated a protrusion in the gastric wall with erosion of the surface epithelium. Further investigations using a computed tomography scan revealed that the protrusion was in fact a submucosal lipoma, which required surgical intervention. This case has shown that a patient presenting with upper gastrointestinal bleeding requiring urgent endoscopic examination may also benefit from further investigations such as a computed tomography scan to confirm the diagnosis