UNASSIGNED: Dengue fever is a mosquito-borne infectious disease endemic in over 100 countries around the world. Among the complications that dengue can cause the Hemophagocytic Lymphohistiocytosis is one of great concern for its severity and complex diagnosis.
UNASSIGNED: Hereby we document a case of this disease expressed on a previously healthy 6-year-old female patient whose dengue infection was so severe that needed intensive care management with vasoactive drugs and diuretics. After a short period of wellness began newly with fever, pancytopenia, hepatitis, and inflammatory response symptoms.
UNASSIGNED: A Dengue associated Hemophagocytic Lymphohistiocytosis syndrome was suspected and treated with intravenous corticosteroids on a 3-day scheme at no signs of malignancy with excellent response. The health care professionals must know about this not novel entity in order to reach an efficient diagnosis and treatment mostly, but not only, those in tropical and sub-tropical regions of the word were dengue virus is endemic.
UNASSIGNED: La fiebre por dengue es una enfermedad infecciosa transmitida por mosquitos, endémica en más de 100 países alrededor del mundo. La Linfohistiocitosis Hemofagocítica, dentro de las complicaciones que puede ocasionar el dengue, es una de las más preocupantes por su complejidad diagnostica y gravedad.
UNASSIGNED: Femenino de 6 años de edad, previamente sana, cuya infección por dengue fue tan grave que requirió manejo en cuidados intensivos. Después de un breve período de bienestar recrudeció la fiebre, además de pancitopenia, hepatitis y síntomas de respuesta inflamatoria.
UNASSIGNED: Se sospechó síndrome de Linfohistiocitosis Hemofagocítica asociada a Dengue y se trató con corticoides intravenosos en un esquema de 3 días con excelente respuesta. Los profesionales de la salud deben conocer esta entidad no novedosa para poder llegar a un diagnóstico y tratamiento eficaz en su mayoría, pero no solo, en las regiones tropicales y subtropicales del mundo donde el virus del dengue es endémico.

Primary splenic angiosarcoma is a malignant vascular neoplasm with a short-term fatal prognosis in most cases, with nonspecific symptoms and usually in advanced stages. We present the case of a 49-year-old man with clinical history of chronic diarrhea and weight loss, in which it was identifies splenomegaly. The histopathological study disclose the diagnosis of primary angiosarcoma of the spleen, with spread to the liver, lung, and lymph nodes. The patient was discharged after undergoing splenectomy, with palliative treatment. Because the rarity of this neoplasm, there is limited experience regarding the best management with a significant impact on patient survival.
El angiosarcoma esplénico primario es una neoplasia vascular maligna con un pronóstico mortal a corto plazo en la mayoría de los casos, que se presenta con síntomas inespecíficos y usualmente en estadios avanzados. Presentamos el caso de un hombre de 49 años, con historia de diarrea crónica y pérdida de peso, en el que se identificó esplenomegalia. El estudio patológico reveló el diagnóstico de angiosarcoma primario de bazo, con diseminación en hígado, pulmón y ganglios linfáticos. Debido a la rareza de esta neoplasia existe poca experiencia en relación con el mejor manejo con impacto significativo en la supervivencia de los pacientes.

OBJECTIVE: Since enzyme replacement treatment (ERT) with idursulfase is available for Hunter syndrome (HS; mucopolysaccharidosis type II), for the first time, disease progression can be limited and organ damage reduced or prevented.
METHODS: We described retrospectively the clinical evolution of eight HS males, treated with ERT and followed in routine clinical practice in Hospital Infantil La Fe (Valencia, Spain).
RESULTS: We studied three children, three adolescents and two adults. Time from diagnosis to ERT ranged from 13.7 to 0.2 years, and duration of ERT ranged from 24 to 77.1 months. From the start of ERT, weight and height increased in children and adolescents and remained stable in adults. Glycosaminoglycans (GAG) decreased in all patients; in patient 5 (aged 23 years), we observed the highest reduction (86%) with recovery of carpal tunnel syndrome, splenomegaly and a decrease in nocturnal oxygen dependence.
CONCLUSIONS: Our results show that ERT improve respiratory impairment and organomegalies and decrease GAGs levels in all patients including children, adolescent and adults. While cardiac manifestations and facial features stabilized, responses in other parameters were heterogeneous.

We describe the case of a 51-year-old woman with a seropositive, erosive, and non-nodular rheumatoid arthritis of 15 year of evolution. The patient had poor compliance with medical visits and treatment. She came to the clinic with persistent pancytopenia and spleen and liver enlargement. Liver and bone marrow biopsies were carried out and amyloidosis, neoplasias and infections were ruled out. We discuss the differential diagnosis of pancytopenia and spleen and liver enlargement in a long-standing rheumatoid arthritis patient.