Spontaneous vulvar hematoma as a rare manifestation of congenital hypofibrinogenemia. case report

ABSTRACT Introduction: Congenital fibrinogen disorders are rare conditions in which there are quantitative and qualitative alterations of factor I; the vast majority of patients are asymptomatic. Case presentation: A 19-year-old female patient with a history of congenital hypofibrinogenemia presented with spontaneous vulvar hematoma along with hypotension, tachycardia, stupor and hematoma of 20cm in the right labium majus. On admission, the young woman had hemoglobin 6.6 g/dL, fibrinogen 74 mg/dL and prolonged clotting times. She received red blood cells transfusion and cryoprecipitates, followed by surgical drainage and intravenous fibrinogen replacement, adjusting the dose according to fibrinogen levels in plasma. The patient presented progressive improvement without hemorrhagic recurrence and fibrinogen levels within the target values until hospital discharge. Discussion: Afibrinogenemia and hypofibrinogenemia are part of the quantitative factor I disorders; in the first case, there is total absence of circulating fibrinogen, and in the second case the levels are below 150 mg/dL. Spontaneous vulvar hematoma as a severe hemorrhagic manifestation is not frequent in symptomatic patients; its treatment is based on fibrinogen replacement in an individualized manner and surgical management when required. Conclusion: Hypofibrinogenemia is a rare disease, and fibrinogen replacement is one of the mainstays of treatment.

RESUMEN Introducción. Los trastornos congénitos del fibrinógeno son una rara condición donde se presentan alteraciones cuantitativas y cualitativas del factor I, siendo asintomáticos la gran mayoría de pacientes. Presentación del caso. Paciente femenino de 19 años con antecedente de hipofibrinogenemia congénita, quien cursa con hematoma espontáneo en vulva y presenta hipotensión, taquicardia, estupor y hematoma de 20cm en labio mayor derecho. Al ingreso, la joven registra hemoglobina 6.6 g/dL, fibrinógeno 74 mg/dL y prolongación de tiempos de coagulación. Se transfunden glóbulos rojos y crioprecipitados; luego se hace drenaje quirúrgico y reposición de fibrinógeno ajustando dosis acorde a fibrinógeno plasmático. La paciente presenta mejoría progresiva sin recurrencia hemorrágica y niveles de fibrinógeno en metas hasta egreso hospitalario. Discusión. La afibrinogenemia y la hipofibrinogenemia hacen parte de los trastornos cuantitativos del factor I, con ausencia total para la primera y niveles <150 mg/dL para la segunda. El hematoma espontáneo de vulva como manifestación hemorrágica severa no es una presentación habitual en pacientes sintomáticos; su tratamiento se basa en la reposición de fibrinógeno de forma individualizada y manejo quirúrgico cuando sea requerido. Conclusión. La hipofibrinogenemia es una enfermedad rara, donde el reemplazo de fibrinógeno es uno de los pilares de tratamiento.

Mucositis bucal en niños con leucemia linfoblástica aguda que reciben quimioterapia en el Hospital Edgardo Rebagliati Martins, Lima-Perú / Oral mucositis in children receiving chemotherapy for acute lymphoblasticleukemia in Edgardo Rebagliati Martins Hospital, Lima-Peru

Determinar la prevalencia de mucositis bucal en niños con leucemia linfoblástica aguda que reciben quimioterapia en el Hospital Edgardo Rebagliati Martins 2007-2011. Materiales y Métodos: Se realizó una investigación retrospectiva de los años 2007 al 2011. El estudio estuvo constituido por las historias clínicas de pacientes con diagnóstico de leucemia linfoblástica aguda del Servicio de Hematología Pediátrica de 1 a 14 años y 11 meses. Los datos fueron recogidos en una ficha elaborada para ser analizados en una base de datos creadas para este fin. Resultados: De las 107 historias clínicas de pacientes con diagnóstico de leucemia linfoblástica aguda, 49 (45.8%) desarrollaron mucositis bucal. De estos 49 pacientes, corresponden al sexo masculino 25 (23.4%) y al femenino 24 (22.4%). El grado 1 de mucositis bucal se presentó con mayor frecuencia en el grupo de 1 a 5 años con 22 (84.6%) casos. El mayor porcentaje de neutropenia y leucocitosis se asoció a la leucemia linfoblástica aguda B en niños con un total de 57.9% y 57.5% respectivamente. Las enfermedades bucales asociadas a la quimioterapia con mayor frecuencia fueron aftas y candidiasis. Conclusión: La prevalencia de mucositis bucal es mayor en niños con leucemia linfoblástica aguda B en el grupo de 1 a 5 años...

To determine the prevalence of oral mucositis in children receiving chemotherapy for acute lymphoblastic leukemia in Edgardo Rebagliati Martins hospital, from 2007 to 2011. Materials and methods: A retrospective investigation was performed between 2007 y 2011. The study was comprised of the medical records of patients with acute lymphoblastic leukemia in the pediatric hematology department from 1 to 14 years and 11 month old. Data were collected on a card to be analyzed in a database created for this purpose. Results: Of the 107 medical records of patients with acute lymphoblastic leukemia, 49 (45.8%) developed oral mucositis. Of these 49 patients, correspond to males 25 (23.4%) and females 24 (22.4%). The oral mucositis grade 1 22 (84.6 %) was more frequently presented in the 0 to 5 years with 22 (84.6%) cases. The highest percentage of neutropenia and leukocytosis was associated with B acute lymphoblastic leukemia in children with an absolute of 92.5% and 91.3% respectively. Conclusion: The prevalence of oral mucositis is higher in children with acute lymphoblastic leukemia B, in the group of 1 -5 years...

Methyl sulfone induces loss of metastatic properties and reemergence of normal phenotypes in a metastatic cloudman S-91 (M3) murine melanoma cell line.

BACKGROUND: The most deadly form of cancer is not lung or colon, breast or prostate; it is any cancer that has become metastatic. Mortality due to metastatic melanoma, one of the most aggressive and deadly cancers, has increased steadily over the last several decades. Unfortunately, the arsenal of chemotherapeutic agents available today is most often unsuccessful at extending and improving the life expectancy of afflicted individuals. We sought to identify an effective and nontoxic agent against metastatic melanoma. METHODOLOGY/PRINCIPAL FINDINGS: We chose to study Cloudman S-91 mouse melanoma cells (sub-clone M3, CCL53.1) because these cells are highly aggressive and metastatic, representing one of the deadliest types of cancer. Melanoma cells also had an experimental advantage because their morphology, which is easily monitored, relates to the physiology of metastatic cells and normal melanocytes. We chose to test methyl sulfone as a chemotherapeutic agent for two reasons. Because of its chemical structure, we speculated a potential anti-cancer activity by targeting microtubules. Equally important, methyl sulfone has a well-established safety profile in humans. Surprisingly, we found that malignant melanoma cells exposed to methyl sulfone demonstrated the loss of phenotypes characteristic of malignant cells, and the reemergence of phenotypes characteristic of healthy melanocytes. Briefly, over time methyl sulfone induced contact inhibition, loss of ability to migrate through an extracellular matrix, loss of anchorage-independent growth, proper wound healing followed by contact inhibition, irreversible senescence followed by arborization with melanosomes in arbors as seen in normal melanocytes. CONCLUSIONS/SIGNIFICANCE: Methyl sulfone may have clinical potential as a non-toxic agent effective against metastatic melanoma. Additionally, methyl sulfone has promise as a tool to explore molecular mechanisms of metastatic transformation as well as fundamental processes such as cell migration, contact inhibition, wound healing and cellular senescence.

Synthesis and evaluation of NO-release from symmetrically substituted furoxans.

A series of symmetrically substituted dibenzoyl furoxans were synthesized and investigated for their potential to release nitric oxide, which plays a key role in the nervous and cardiovascular systems. Cysteine was employed to promote nitric oxide release from furoxan via the formation of an S-nitrosothiol intermediate. Transition metal ion-mediated S-nitrosocysteine decomposition liberates nitric oxide that, in aqueous aerobic solutions, is converted to reactive nitrogen oxide species. The percent nitric oxide released was quantified colorimetrically by the Griess reagent system.