Newly diagnosed adult AML and MPAL patients frequently show clonal residual hematopoiesis.

Adult acute myeloid leukemia (AML) is a highly heterogeneous stem cell malignancy characterized by the clonal expansion of immature myeloid precursors. AML may emerge de novo, following other hematopoietic malignancies or after cytotoxic therapy for other disorders. Here, we investigated the clonal vs reactive nature of residual maturing bone marrow cells in 59 newly diagnosed adult AML and mixed phenotype acute leukemia (MPAL) patients as assessed by interphase fluorescence in situ hybridization analysis of AML and myelodysplastic syndrome-associated cytogenetic alterations and/or the pattern of chromosome X inactivation, in females. In addition, we investigated the potential association between the degree of molecular/genetic involvement of hematopoiesis and coexistence of altered immunophenotypes by flow cytometry. Our results indicate that residual maturing neutrophils, monocytes and nucleated red cell precursors from the great majority of newly diagnosed AML and MPAL cases show a clonal pattern of involvement of residual maturing hematopoietic cells, in association with a greater number of altered immunophenotypes. These findings are consistent with the replacement of normal/reactive hematopoiesis by clonal myelopoiesis and/or erythropoiesis in most newly diagnosed AML and MPAL cases, supporting the notion that in most adults presenting with de novo AML, accumulation of blast cells could occur over a pre-existing clonal hematopoiesis.

Rotura de la arteria axilar en un caso de síndrome de Ehler-Danlos tipo IV / Axillary artery rupture in a case of Ehler-Danlos type IV syndrome

El síndrome de Ehler-Danlos (SED) tipo IV o síndrome de Sacks-Barabas es una enfermedad del tejido conectivo altamente infrecuente. El diagnóstico clínico se basa en la presencia de, al menos, dos de los cuatro criterios diagnósticos mayores, y se confirma por la demostración de una estructura anormal de las moléculas de procolágeno tipo III o la presencia de una mutación en el gen COL3A1. Las complicaciones vasculares pueden afectar a todas las áreas anatómicas, con tendencia a incluir a las arterias de diámetro grande y mediano. Las lesiones vasculares proximales del miembro superior pueden conducir a la pérdida de la extremidad, y con gran frecuencia se asocian a lesiones nerviosas. Se presenta el caso de un niño de 13 años que comenzó con rotura no traumática de la arteria axilar, y se hace una revisión bibliográfica de esta enfermedad

Ehler-Danlos syndrome (EDS) type IV or Sacks-Barabas syndrome is a rare disorder in the connective tissue. Its clinical diagnosis is based on at least two of four major diagnostic criteria and it is confirmed by the demonstration of structurally abnormal type III procollagen molecules or the presence of mutation in the COL3A1 gene. Vascular complications may involve all the anatomical areas, with a tendency to include large and medium diameter arteries. Proximal vascular injuries in the upper limb may lead to amputation and are often associated to neurologic injuries. The clinical case of a 13-year-old-boy, who started with a non-traumatic rupture of the axillary artery is presented. The main aspects on the disease presented in the literature are reviewed

Rotura de la arteria axilar en un caso de síndrome de Ehler-Danlos tipo IV / Axillary artery ruptura in a case of ehler-danlos type IV syndrome

El síndrome de Ehler-Danlos (SED) tipo IV osíndrome de Sacks-Barabas es una enfermedad del tejido conectivo altamente infrecuente. El diagnóstico clínico se basa en la presencia de, al menos, dos de los cuatro criterios diagnósticos mayores, y se confirma por la demostración de una estructura anormal de las moléculas de procolágeno tipo III o la presencia de una mutación en el gen COL3A1.Las complicaciones vasculares pueden afectar a todas lasáreas anatómicas, con tendencia a incluir a las arterias de diámetro grande y mediano. Las lesiones vasculares proximales del miembro superior pueden conducir a la pérdida de la extremidad, y con gran frecuencia se asocian a lesiones nerviosas.Se presenta el caso de un niño de 13 años que comenzócon rotura no traumática de la arteria axilar, y se hace una revisión bibliográfica de esta enfermedad

Ehler-Danlos syndrome (EDS) type IV orSacks-Barabas syndrome is a rare disorder in the connective tissue. Its clinical diagnosis is based on at least two of four major diagnostic criteria and it is confirmed by the demonstration of structurally abnormal type III procollagen molecules or the presence of mutation in the COL3A1 gene.Vascular complications may involve all the anatomical areas, with a tendency to include large and medium diameter arteries. Proximal vascular injuries in the upper limb may lead to amputation and are often associated to neurologic injuries.The clinical case of a 13-year-old-boy, who started with a non-traumatic rupture of the axillary artery is presented. The main aspects on the disease presented in the literature are reviewed

Tratamiento de atelectasias postoperatorias mediante fibrobroncoscopia asociada a Manujet / No disponible

No disponible

Espondilodiscitis tuberculosa lumbar / Tuberculous lumbar discospondylitis

La espondilodiscitis tuberculosa constituye la localización más frecuente de la infección tuberculosa del aparato locomotor. La zona más frecuentemente afectada en la columna es la región dorsal. La resonancia magnética nuclear (RMN) es el método de imagen de elección en el diagnóstico de la espondilodiscitis, especialmente en las fases tempranas. La espondilodiscitis tuberculosa, especialmente en la columna lumbar, puede ser tratada de forma satisfactoria de forma conservadora y hay pocas indicaciones para el tratamiento quirúrgico. Se presenta el caso de una mujer de 53 años con afectación de la columna lumbar. La paciente presentaba dolor lumbar de carácter inflamatorio de un año de evolución. El Mantoux fue positivo. En la RMN presentaba una imagen compatible con espondilodiscitis L4-L5 y la gammagrafía era sugestiva de proceso infeccioso activo. Realizó tratamiento médico con una evolución favorable (AU)

Amiloidosis asociada a hemodiálisis como causa de subluxación atlo-axoidea anterior / Amyloidosis associated to hemolialysis as casuse of anterior atlo-axoid subluxation

La amiloidosis por depósito de beta 2-microglobulina constituye una complicación frecuente en los pacientes con insuficiencia renal crónica tratados con hemodiálisis durante largos períodos de tiempo. Se caracteriza por una alta incidencia de afectación poliarticular, desarrollando una espondiloartropatía destructiva que afecta predominantemente a la columna cervical, y que en casos graves puede provocar una subluxación atlo-axoidea. Se presenta el caso clínico de un paciente varón de 54 años de edad afectado de amiloidosis asociada a hemodiálisis, que presentó dolor cervical y rigidez resistente a tratamiento conservador. Tras realizar una radiografía lateral de columna cervical en máxima flexión y TAC cervical, se diagnosticó una subluxación atlo-axoidea anterior. El paciente fue intervenido quirúrgicamente, estando actualmente asintomático (AU)

Stat3 is present in the developing and adult rat cerebellum and participates in the formation of transcription complexes binding DNA at the sis-inducible element.

Stat3 is a member of a recently identified family of proteins named STATs for their ability to act as signal transducers and activators of transcription. Stimulation of epidermal growth factor or cytokine receptors can cause activation of Stat3 and transduction of specific responses to the nucleus. Here the presence of Stat3 has been examined in the rat cerebellum at different times during postnatal development and adulthood by means of immunohistochemistry and western blotting. In addition, DNA binding activity at the sis-inducible element that is present in the promoter of the c-fos gene has been studied by electrophoretic mobility shift assay. The results have shown that Stat3 p92 is abundant in the rat cerebellum. Stat3 was found in the external granule cell layer and also within the molecular layer from postnatal day (P) 0 to P7. From P15 to the adult, the internal granule cell layer and Purkinje cells were also stained for Stat3. Nuclear extracts were found to contain DNA binding activity to the sis-inducible element during development and adulthood. Supershift assays demonstrated that Stat3 mediates the formation of one protein-DNA complex. The present results suggest that Stat3 participates in intracellular signaling and is involved in maintaining basal c-fos expression in the cerebellum of the developing and adult rat under physiological conditions.

Induction of Stat3, a signal transducer and transcription factor, in reactive microglia following transient focal cerebral ischaemia.

Stat3, a member of the family of cytoplasmic signal transducers and activators of transcription, was found in the rat brain in vivo under physiological conditions and was stimulated following transient focal cerebral ischaemia. A transient episode of middle cerebral artery occlusion induced a strong microglial response in the areas undergoing neural cell death from 4 days after middle cerebral artery occlusion. This was accompanied by increased expression of Stat3 in the ipsilateral cortex and striatum, as revealed by Western blotting of tissue extracts. Immunohistochemistry showed strong induction of Stat3 in reactive microglial cells 4, 7 and 15 days after cerebral ischaemia. Stat3 was seen in the microglia cytoplasm, but in many microglial cells immunoreactivity was also distributed within the nucleus. These results suggest that Stat3 mediates signal transduction and activates transcription in reactive microglia in vivo following brain ischaemia.

Transforming growth factor-alpha (TGF-alpha) and epidermal growth factor-receptor (EGF-R) immunoreactivity in normal and pathologic brain.

Transforming growth factor alpha (TGF-alpha) and epidermal growth factor-receptor (EGF-R) immunoreactivity is observed in the majority of neurons, and in maturing astrocytes, in the developing and adult brain of humans and different species of animals. TGF-alpha and EGF-R co-localize in most neurons and maturing astrocytes, suggesting that most TGF-alpha-producing cells are EGF-R-expressing cells. TGF-alpha and EGF-R immunoreactivity decrease in damaged areas following different insults. However, EGF-R appears in reactive glia, mostly reactive astrocytes, within and surrounding the damaged areas. TGF-alpha and EGF-R immunoreactivity is found in neurons of patients affected by Alzheimer's disease and other forms of dementia, and in neurons of patients suffering from epilepsy owing to different causes, thus pointing to the conclusion that TGF-alpha does not play a significant role in these pathologies. However, EGF-R immunoreactivity occurs in reactive astrocytes and microglia in subacute but not chronic lesions in human cases. Since TGF-alpha is a membrane-anchored growth factor, which may be cleaved leading to the formation of soluble forms, and both the membrane-anchored and soluble forms have the capacity to activate the EGF-R, it is feasible that TGF-alpha in the nervous system may act upon EGF-R-containing neurons through different mechanisms. In addition to distant effects resulting from the release of soluble TGF-alpha, local effects may be produced by establishing direct cell-to-cell contacts (juxtacrine stimulation), or in cells expressing both TGF-alpha and EGF-R (autocrine stimulation).