Dermatomiositis asociada a cáncer de mama. Síndrome paraneoplásico infrecuente / Dermatomyositis associated with breast cancer. An infrequent paraneoplastic syndrome

La dermatomiositis es un síndrome paraneoplásico raro que se asocia al diagnóstico de diferentes tumores. El cáncer de mama es un tumor asociado de manera muy infrecuente con la dermatomiositis. Se presenta el caso de una mujer de 50 años que comenzó como una dermatomiositis y que fue diagnosticada de un cáncer de mama. El tratamiento quirúrgico del cáncer de mama supuso la desaparición de los síntomas de debilidad muscular y de las alteraciones cutáneas(AU)

Dermatomyositis is a rare paraneoplastic syndrome associated with several malignant tumors. The association with breast cancer is much less frequent. We report a 50-year-old woman who presented with dermatomyositis and was diagnosed with breast cancer. Surgical treatment of breast cancer improved the symptoms of muscular weakness and the cutaneous manifestations of dermatomyositis(AU)

Parasitemia with plasmodium falciparum in a patient after cardiac surgery in a non-endemic country

We present a case-report of a febrile patient after cardiac surgery; the patient had no history of travel to areas where malaria is endemic. After several studies and due to the background of blood transfusion, blood smear, serology and PCR were done and revealed the presence of P. falciparum; 14 donors were associated with the transfusions; none had travelled to malaria areas; serology, PCR and chromatography were negative in 13 (1 refused testing); no cases of post-transfusion malaria were reported in the region. Transmission of malaria infection in the reported case remains unknown but might be linked to blood transfusion, although malariadue to establishment of Plasmodium sp. in indigenous or aircraft-travelling mosquito remainsa possibility. Increases in international exchanges from endemic areas enhance the possibility that blood donors might have been in contact with malaria. Accurate histories of potential exposure to blood transfusions are necessary in the work-up of febrile patients (AU)

Se presenta el caso de un paciente sin antecedente de viajes internacionales recientes que presentó fiebre tras cirugía cardiaca. Tras varias pruebas complementarias y dado el antecedente transfusional se realizaron una tinción de Giemsa de sangre periférica, serología y PCR que fueron positivas para P. falciparum. La investigación de los 14 donantes fue negativa (no antecedente de estancia en zona endémica para malaria; serología, PCR y cromatografía negativas en 13 –un donante se negó a más estudios); no se comunicaron otros casos de malaria post-transfusional en la región. Aunque no se pudo confirmar el origen de la infección podría estar relacionada con la transfusión, sin poder descartar transmisión local en zona noendémica por mosquitos autóctonos o “malaria de los aeropuertos”. Los movimientos migratorios aumentan el riesgo de exposición a Plasmodium spp. entre donantes de sangre. En el estudio de un paciente con fiebre se debe valorar el antecedente de transfusión e incluir la malaria en el diagnóstico diferencial (AU)

HLA-DR antibodies in transfusion-related acute lung injury (TRALI): a case report.

Transfusion-related acute lung injury (TRALI) is a serious adverse consequence of blood product transfusion. Cases of TRALI have gone unrecognized or misdiagnosed, since the symptoms can be confused with other transfusion-related events or with non-transfusion related comorbidities. Suspected cases of TRALI may be insufficiently investigated, and mild or moderate cases may not be investigated or reported at all. We report here the case of a 73-year man who developed TRALI following a transfusion of packed red blood cells (pRBCs) mediated by HLA class II antibodies (HLA-DR) detected by luminex technology. A very few cases of TRALI have been described being caused by HLA class II antibodies without the simultaneous presence of anti-HLA class I antibodies. Technology for antibody detection has increased the power and the specificity, especially with the use of flow cytometry with a better definition of the antigen/antibody pairs that have resulted in TRALI episodes. In this sense, HLA class II antibodies can exactly be detected with these methods and have surely been underestimated until now.

Inhibition of proteasome by bortezomib causes intracellular aggregation of hepatic serpins and increases the latent circulating form of antithrombin.

Conformational diseases include heterogeneous disorders sharing a similar pathological mechanism, leading to intracellular aggregation of proteins with toxic effects. Serpins are commonly involved in these diseases. These are structurally sensitive molecules that modify their folding under even minor genetic or environmental variations. Indeed, under normal conditions, the rate of misfolding of serpins is high and unfolded serpins must be degraded by the proteasome system. Our aim was to study the effects of bortezomib, a proteasome inhibitor, on conformationally sensitive serpins. The effects of bortezomib were analysed in patients with multiple myeloma, HepG2 cells, and Swiss mice, as well as in vitro. Levels, anti-FXa activity, heparin affinity, and conformational features of antithrombin, a relevant anticoagulant serpin, were analysed. Histological, ultrastructural features and immunohistological distribution of antithrombin and alpha1-antitrypsin (another hepatic serpin) were evaluated. We also studied the intracellular accumulation of conformationally sensitive (fibrinogen) or non-sensitive (prothrombin) hepatic proteins. The inhibition of the proteasome caused intracellular accumulation and aggregation of serpins within the endoplasmic reticulum that was associated with confronting cisternae and Mallory body formation. These effects were accompanied by a heat stress response. Bortezomib also increased the levels of intracellular fibrinogen, but has no significant effect on prothrombin. Finally, bortezomib had only minor effects on the mature circulating antithrombin, with increased amounts of latent antithrombin in plasma. These results suggest that the impairment of proteasomal activities leads to an intracellular accumulation of conformationally sensitive proteins and might facilitate the release of misfolded serpins into circulation where they adopt more stable conformations.