Independent validation of the adjusted GAPSS: Role of thrombotic risk assessment in the real-life setting.

Objective The objective of this report is to independently validate the adjusted Global Antiphospholipid Syndrome Score (aGAPSS) to predict thrombosis in a cohort of patients with APS and/or autoimmune disease. Methods This retrospective cohort study included 319 consecutive patients with APS and/or autoimmune disease. Data on clinical manifestations, conventional cardiovascular risk factors and aPL profile were collected. The aGAPSS was calculated for each patient by adding together the points corresponding to the risk factors. Results Among the 319 patients included (mean age: 48.0; SD 15.47), conducted over a mean period of 52 months (range: 19-394), 219 fulfilled the current APS classification criteria (PAPS diagnosed in 130 patients and APS associated autoimmune disease (aAPS) in 89 patients), and 100 patients with autoimmune disease without APS (AD). A total of 201 patients (63.0%) had a history of one or several thrombotic manifestations, 189 (86.3%) of them APS patients: 118 PAPS (mean age: 50.14; SD 15.47) and 71 aAPS (mean age: 48.13; SD 15.81). Higher aGAPSS baseline values were seen in patients with thrombosis 6.58 (SD 3.36) when compared with those without 4.90 (SD 4.33) ( p = 0.001). Conclusions This study has shown that even when anti-phosphatidylserine/prothrombin antibodies (aPS/PT) are not computed in an adjusted model of GAPSS (aGAPSS), this score represents an improvement in assessment of the risk prediction of thrombosis in APS patients and/or autoimmune disease. However, cut-off values may differ from other kinds of cohorts, which suggests that baseline characteristics in divergent groups of patients can account for differences in cut-off values of GAPSS.

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.

[Incidence of febrile episodes in patients with multiple myeloma undergoing autologous peripheral blood stem cell transplantation]. / Incidencia de episodios febriles en pacientes con mieloma múltiple sometidos a trasplante autólogo de progenitores hematopoyéticos de sangre periférica (TASPE).

BACKGROUND: Autologous peripheral blood stem cell transplantation (PBSCT) is a procedure frequently used as therapy for hematological malignancies, in which infectious complications are a major cause of morbimortality. The duration and intensity of neutropenia, indwelling central venous catheters, and mucosa chemotherapy-induced damage, contribute to increase infection rates. We have retrospectively review the incidence of febrile episodes and microbiological documented infections (MDI) in patients with multiple myeloma (MM) undergoing PBSCT. PATIENTS AND METHODS: We have retrospectively analyzed 56 PBSCT in patients diagnosed of MM between 1995 and 2002 in our hospital. RESULTS: 34 patients showed fever: 19 fever of unknown origin; 5 clinically documented infections and 10 patients MDI. We isolated 5 pathogens gram negative and 4 gram positive. We observed 2 infections associated to indwelling central venous catheters and 1 MDI due to simplex Herpes Virus. Two patients died due to infectious complications. CONCLUSIONS: The incidence of febrile episodes in our patients is similar to those previously reported as well, duration of neutropenia associated to PBSCT. We have observed a slightly higher incidence of gram negative pathogens.

Incidencia de episodios febriles en pacientes con mieloma múltiple sometidos a trasplante autólogo de progenitores hematopoyéticos de sangre periférica (TASPE) / Incidence of febrile episodes in patients with multiple myeloma undergoing autologous peripheral blood stemm cell transplantation

Introducción: El trasplante autólogo de progenitores hematopoyéticos de sangre periférica (TASPE), es un procedimiento frecuentemente empleado en el tratamiento de las hemopatías malignas, siendo la morbimortalidad atribuible a las infecciones un factor determinante en el resultado final del mismo. La duración e intensidad de la neutropenia, la presencia de catéteres venosos centrales y la alteración de las mucosas contribuyen a una mayor incidencia de infecciones. Revisamos de manera retrospectiva la incidencia de episodios febriles y en especial de infecciones microbiológicamente documentadas (IMD) en pacientes afectos de mieloma múltiple (MM) sometidos a TASPE. Pacientes y métodos: Análisis retrospectivo de una serie de 56 pacientes con MM sometidos a TASPE en nuestro hospital entre 1995 y 2002. Resultados: Treinta y cuatro pacientes presentaron fiebre: 19 episodios febriles sin foco; 5 episodios febriles clínicamente documentados y 10 pacientes IMD. Se aislaron 5 microorganismos gram negativos frente a 4 gram positivos. Se evidenció infección de catéter en 2 casos. Sólo observamos una IMD de etiología vírica por virus Herpes simple (VHS). Dos pacientes fallecieron como consecuencia del proceso infeccioso. Conclusiones: El porcentaje de procesos febriles en nuestra serie es similar al descrito en la literatura, así como la duración de la neutropenia asociada al TASPE. Observamos un discreto predominio de la infecciones por gram negativos

Background: Autologous peripheral blood stem cell transplantation (PBSCT) is a procedure frequently used as therapy for hematological malignancies, in wich infectious complications are a major cause of morbimortality. The duration and intensity of neutropenia, indwelling central venous catheters, and mucosa chemotherapy-induced damage, contribute to increase infection rates. We have retrospectively review the incidence of febrile episodes and microbiological documented infections (MDI) in patients with multiple myeloma (MM) undergoing PBSCT. Patients and methods: We have retrospectively analized 56 PBSCT in patients diagnosed of MM between 1995 and 2002 in our hospital. Results: 34 patients showed fever: 19 fever of unknown origin; 5 clinically documented infections and 10 patients MDI. We isolated 5 pathogens gram negative and 4 gram positive. We observed 2 infections associated to indwelling central venous catheters and 1 MDI due to simplex Herpes Virus. Two patiens died due to infectious complications. Conclusions: The incidence of febrile episodes in our patients is similar to those previously reported as well, duration of neutropenia associated to PBSCT. We have observed a slightly higher incidence of gram negative pathogens

Genética de las sobrecargas y el síndromecongénito de hiperferritinemia y cataratas / Genetics of iron overloads and hereditary hyperferritinemia cataract syndrome

No disponible

Falsa anemia por crioaglutininas precedida en el tiempo de una anemia hemolítica autoinmune por anticuerpos calientes / False anemia preceeding in time by an autoimmune hemolytic anemia by warm antibodies

No disponible

[Respiratory distress syndrome due to hyperleukocytic leukemias]. / Síndrome de distrés respiratorio por leucemias hiperleucocíticas.

Hyperleukocytic leukemias are a small proportion of leukemias that have white blood cell count > 100 x 10(9)/l, most of them are leukemic blast cells. These leukemias have a grave prognosis because they can develop a leukostasis syndrome which describes: the acute onset of pulmonary failure and, often, neurologic deficits and disseminated intravascular coagulation (DIC). The leukostasis is produced by the mechanical obstruction of vascular bed by blast cells, which can be induced by the spontaneous tumor lysis or as a side effect of cytotoxic drugs. So, hyperleukocytic leukemias require early and vigorous measures to decrease the white blood cell count, using leukapheresis and/or chemotherapy, before pulmonary failure exists. Then, it is possible to reverse the lesions. We report two cases of acute myeloblastic leukemia with a white blood count > 100 x 10(9)/l, that developed a respiratory distress syndrome and died. The postmortem examination has been done in one of the cases.

Síndrome de distrés respiratorio por leucemias hiperleucocíticas / Respiratory distress syndrome due to hyperleukocytic leukemias

Las leucemias hiperleucocíticas constituyen una pequeña proporción de leucemias que debutan con un recuento leucocitario superior a 100 x 109/l, blastos leucémicos en su mayoría. Tienen un mal pronóstico ya que en esta situación se puede desarrollar un síndrome de leucostasis que consiste en una insuficiencia respiratoria aguda a menudo acompañada de alteraciones del sistema nervioso central y de la coagulación, debido a una leucostasis por ocupación de los vasos por blastos como consecuencia de una lisis tumoral espontánea o como efecto secundario de los fármacos citotóxicos. Así pues, ante una leucemia con hiperleucocitosis se debe de instaurar un tratamiento precoz y enérgico como la leucoaféresis y/o la quimioterapia para disminuir el recuento leucocitario antes de la aparición de síntomas respiratorios. Ya que si se reduce rápidamente el recuento leucocitario existe la posibilidad de reversibilidad de las lesiones. Presentamos dos casos de leucemia aguda mieloblástica con un recuento leucocitario superior a 100 x 109/l que desarrollaron un síndrome de distrés respiratorio de instauración aguda y evolución fatal. Habiéndose realizado valoración anatomopatológica en uno de ellos (AU)

[Anti-A antibodies and bacterial contamination of platelet concentrates]. / Anticuerpos anti-A y contaminación bacteriana de los concentrados de plaquetas.

OBJECTIVES: The possible ABO group antibodies protective function against several infections has been classically described. We analyze the platelet concentrates (PC) bacterial control results and their ABO antibodies. MATERIAL AND METHODS: We studied 245 outdated PCs (> 5 days). The samples were sterilely collected for adequate microbiological investigation studies on sheep-blood agar plates. If bacterial growth is found, the microbiological identification is performed on the basis of standard tests, the specific anti-biotype being achieved by disk-diffusion method on Müeller-Hinton agar plates, and the red cell concentrate was analyzed. RESULTS: Bacterial growth by negative coagulase Staphylococcus was found in 10 PCs (4.1%; CI95%; 1.97-7.37). The contaminated PCs lacked natural anti-A antibodies. There were no statistical differences when we analyzed the PC's age, colour or blood group. COMMENTS: The anti-A antibodies may be a protective factor versus PCs contamination caused by resident bacteria.

[Transfusion and pre-deposit autotransfusion in orthopedic and traumatologic surgery]. / Transfusión y autotransfusión predepósito en cirugía ortopédica y traumatológica.

PURPOSE: We present a retrospective study about the transfusional needs and the results of the preoperative autologous donation programme in our centre during 1996, in the Orthopaedic and Traumatologic Surgery Service's patients (SOT). PATIENTS AND METHODS: During 1996 the SOT Service carried out 592 major programmed surgical procedures with implants: hip prostheses (HP), knee prostheses (KP) and spinal column fixings (SCF). We've reviewed transfusion forms of the Blood Bank and the preoperative autologous donation forms of the patients sent to us. We've analysed the data with the EpiInfo 6.04 of CDC. Atlanta and SSPS programmes, using the chi 2 of Pearson's Test for qualitative variables and T-Student's test for 2 quantitative variables. RESULTS: The put 387 HP, 118 KP and 87 SCF. 1399 units of blood were administered to 437 patients: 310 HP, 64 KP and 63 SCF. The units/patient (U/P) mean was: 3.3 in HP, 2.3 in PTR and 3.5 in SCF. They sent 99 patients to be programmed for autotransfusion, of which 15 were excluded. The patients' number and the surgical procedures were: 39 HP, 7 KP and 38 SCF. They solicited 247 units (2.9 U/P) and we took out 91.4% of these and the programme was finished in the 73.8% of patients. They transfused 311 U, autologous 176, to 91.6% of programmed patients. Only autologous blood was received by 48% of transfused patients without any statistically significant differences (SSD) (p > 0.05) between the different prostheses. The difference among the transfusion needs of HP and SCF and over KP is SSD (p < 0.001), being non significant between HP and SCF. The probability of getting an autotransfusion programme is bigger in SCF, with a SSD (p < 0.001) of SCF over the HP and the KP and non significant between the last two. The probability of being transfused is greater in the programmed autotransfusion group, this being SSD (p < 0.001), as much of global form as comparing separately each type of prostheses. In the programmed autotranfusion group there are no SSD (p > 0.05) between the type of prostheses and the probability of being transfused, finding the same SSD when we compare the U/P mean and each type of prostheses. The opposite occurred to the patients programmed, it is SSD (p < 0.001) as much of global form as comparing the U/P means in each type of prostheses. Finally, between the global U/P transfused global in the patients that we did an autotransfusion on and those we didn't the difference is SSD (p < 0.001), seeing the same SSD comparing the U/P means in each type of prostheses between both groups. CONCLUSIONS: Given that the autotransfusion is safer than the homologous transfusion and the high percentage of this type of patients that need transfusion, 80% of HP, 54% of KP and 73% of SCF 72%, it would be desirable to enlarge the autotransfusion preoperative programme because it is only carried out on 14.18% of this type of procedures.