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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematodermic neoplasm usually involving the skin. In this retrospective case series, 10 cases of BPDCN were identified, 90% of which had skin involvement and exhibited predominantly violaceous nodules and/or bruise-like plaques. Skin lesions showed diffuse or nodular dermal-based infiltrates of intermediate sized blasts with a grenz zone. Tumor immunophenotyping was CD4(+), CD56(+), CD123(+) and CD303(+). The most frequently mutated genes according to targeted next-generation sequencing were TET2 (3/7) and NRAS (2/7). Multiagent chemotherapy (CT) was administered as first-line therapy, and a total of 5 patients underwent allogenic hematopoietic stem cell transplantation (allo-HSCT). Better outcomes were observed in younger patients and those treated with acute lymphoblastic leukemia (ALL)-like CT followed by allo-HSCT. This study shows the clinical range of cutaneous lesions of BPDCN. Despite the absence of a gold standard therapy, patients treated with myeloablative intensive regimens and allo-HSCT seem to have a more favorable prognosis., (Copyright © 2024 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

We carry out a brief analysis of the constitutional grounds on health protection (arts. 43 in connection with 15 of the Spanish Constitution [CE]), in the sense that we don't need to consider exogenous principles to a normative constitution as the Spanish one, such as the known and cited principle of necessity, under the aphorism Salus publica suprema lex esto. We find in the constitutional text itself the attribution to the public powers of the competence to organize and protect public health (art. 43 CE), therefore public powers are able to regulate by law the duties and obligations of citizens «in cases of serious risk, catastrophe or public calamity» (art. 32(4) CE). Moreover, we study the Spanish legal framework on public health, including the General Sanitary Law, the General Public Health Law, with a special attention to the art. 3 of Organic Law 3/1986, 14 April, on special measures in the field of public health, which attributes to the health authorities a huge competence to be able to adopt, in a situation of sanitary crisis such as the current one, decisions that, with the necessary guarantees, suppose a restriction or limitation of rights, including fundamental ones as in the case of confinement. These limitations on fundamental rights require a judicial authorization or ratification. However, we also consider the convenience of a new organic law to regulate in detail the procedure and the legal guarantees to carry out the adoption and development of these measures., (Copyright © 2020 SESPAS. Publicado por Elsevier España, S.L.U. All rights reserved.)

The authors describe the profile corresponding to students enrolled in first, second and third year courses to become registered nurses in Catalonia, along with their professional and job expectations; the authors examine students' perceptions of the university environment. This information will be a great aid to, on the one hand, update the performances and initiatives taken by those responsible for nursing schools, and on the other hand, to obtain a preliminary view on future nursing professionals. At the same time, this information will provide useful elements for students themselves to reflect on their studies and their future as professionals.

Purpose: To evaluate the increase in contrast sensitivity to intraocular lens TECNIS Z-9000 (Pfizer) compared with other intraocular lenses., Method: We implanted a TECNIS Z-9000 lens in 18 patients who had another type of intraocular lens implanted in their other eye. We then tested contrast sensitivity under both mesopic and photopic conditions using the VCTS (Vision Contrast Test System) provided by Vistech Consultants Inc., Results: There was an improved contrast sensitivity at low and medium frequencies, following TECNIS-Z 9000 lens implantation, however this improvement did not reach statistical significance. There were significant differences at high frequencies, which are directly related to the quality of visual function., Conclusions: TECNIS Z-9000 lens implantation (with a modified anterior surface) achieves a significant improvement in contrast sensitivity, meaning in practice an increase in the quality of visual function.

Background: Autologous stem cell transplantation (ASCT) is increasingly performed on an outpatient basis. Preliminary results show a significant financial saving without modifying the therapeutic results. The aim of this study has been to evaluate the feasibility of this procedure in our the Hospital Clínic of Barcelona, Spain., Patients and Methods: All patients in whom an ASCT due to a hematological malignancy, having a good performance status, a caregiver 24 h per day, a lodging near to the hospital and an appropriate cultural and social environment, were offered an outpatient management starting the day after stem cell administration (day +1)., Results: During the study period (April, 1997-June, 1998) 64 ASCT were performed. Fifteen patients (23%) were managed on an outpatient basis. The remaining patients were not included in the study due to patient preference (26%), medical reasons (22%), distant home (18%) and social problems (9%). Seven patients (47%) had to be readmitted in the hospital due to fever (n = 4), nausea (n = 2) or respiratory distress (n = 1). Patients receiving total body radiation (TBI) in the conditioning regimen did not have a higher readmission rate than those conditioned without TBI. The median hospital stay (since day +1) was 6 (range: 0-22) and 17 (13-117) days for outpatient and inpatients respectively (p = 0.0016). This reduction would imply a saving of 65% in hospitalization charges and of 12% in total charges. All patients having elected the outpatient management were very satisfied by having made that choice., Conclusion: With an appropriate patient selection, outpatient ASCT is a feasible method that may improve the quality of life of the patients and can reduce the costs of the procedure.

A 47 year old woman with a MALT lymphoma affecting the breast exclusively is reported. Complete response was achieved after CHOP chemotherapy but three years later a relapse was observed. Second-line chemotherapy (CNOP) and local radiotherapy were administered, and a second remission was obtained lasting for 1 year at last follow-up. A bibliographic search of MALT lymphomas of the breast showed that most such cases correspond to localized forms (stage I-II) and that from the radiologic point of view nodular lesions are almost the rule, although in the present case diffuse involvement was observed. MALT lymphomas of the breast tend to remain localized, and to relapse locally, and these facts appear as independent of the treatment applied (surgery, radiotherapy, chemotherapy or any combination).

Background: Idiopathic myelofibrosis (IM) is an infrequent myeloproliferative disorder with few large series published in the medical literature. Information on the characteristics of more recently diagnosed patients with IM is scarce., Patients and Methods: The initial features, evolutive patterns and survival from 106 patients diagnosed with IM in a single institution between 1975 and 1996 were analyzed., Results: Median age of the series was 64 years (range: 17-89); there were 61 males and 55 females. One third of the patients were asymptomatic at IM diagnosis. The most common presenting symptoms were related to hypermetabolism, anemia and splenomegaly. A palpable spleen was noted in 85% of patients, and 66% had hepatomegaly. Anemia was the most frequent hematologic abnormality (50% of cases). Bone marrow biopsy showed cellular phase IM in 50 patients, IM without osteosclerosis in 39, and osteosclerotic IM in the remaining 17. Evolution into acute leukemia was seen in 14 patients (actuarial probability at 7 years: 20%, 95% CI: 0-40%), 8 patients developed portal hypertension, 5 liver failure without portal hypertension and 14 heart failure. With 62 patients having died, the series' median survival was 59 months (95% CI: 41-75)., Conclusion: IM is usually diagnosed in the old age. From the histologic point of view, a predominance of the cellular phase is observed at disease presentation. In recent years an increased proportion of patients asymptomatic at diagnosis is observed.

The increase in cancer incidence in renal transplant recipients is a well recognized fact, which has been related to post-transplant immunosuppressive therapy. Solid tumors, skin cancer and non-Hodgkin's lymphomas account for most of the neoplasms in these patients, whereas chronic myeloproliferative disorders are infrequent. A patient is reported who developed chronic myelogenous leukemia (CML) six years after renal transplantation, which was followed by immunosuppressive with azathioprine, and the published data on such an association are reviewed. In all 10 cases reported azathioprine had been administered after transplantation. The amount and type of post-transplant immunosuppressive therapy seems to be the most important risk factor for the development of secondary CML in these patients, since no cases of CML in patients receiving cyclosporine A have been reported.

Background: In the last years the treatment of patients with chronic lymphocytic leukemia (CLL) and low-grade lymphomas (NHL) has changed because of the introduction of new agents, mainly the purine analogs. We report our experience with fludarabine in patients with indolent lymphoid malignancies that were previously treated with conventional agents., Patients and Methods: Twenty patients were studied. Eleven had CLL and nine NHL. Among the patients with CLL and NHL, 72 and 100%, respectively, had advanced disease. All patients had previously been treated. Fludarabine was administered by intravenous infusion at a dose of 25 mg/m2, for 5 days every 4 weeks until a maximum of 6 cycles., Results: Neither complete response (CR) nor partial response (PR) was recorded in patients with CLL; 4 (36%) achieved clinical improvement. Among the 9 patients with NHL, 3 (33%) had a CR and one a PR; two of the 3 patients with CR also achieved a molecular remission. In 3 patients with CLL their disease progressed from stage II to IV. Three patients (one with CLL and two with NHL) developed high-grade lymphoma during or immediately after the treatment with fludarabine. The major toxicities were infections: 3 patients had lobar pneumonia and one an interstitial pneumonia without microbiological identification., Conclusions: Fludarabine is an active agent in patients with low-grade lymphoid malignancies refractory to the treatment or in relapse. The possibility of obtaining molecular remissions makes this agent specially interesting in those therapies including hemopoietic progenitors transplantation as intensification treatment.

The idiopathic hypereosinophilic syndrome (HES) is an infrequent entity defined by an eosinophil count > 1.5 x 10(9)/l and the specific infiltration of different organs not attributable to another disease. Its prognosis is marked by the complications derived from visceral, particularly cardiac, infiltration determining a median survival of less than one year if treatment is not administered. Although a notable prolongation in survival may be achieved in these patients with the administration of glucocorticoids and cytolytic drugs, mainly hydroxiurea, there remains a group of patients who do not respond to these therapeutic measures, thus leading to alpha interferon trials with promising results. A favourable experience with the use of alpha interferon in two patients with IHS resistant to conventional treatment is reported. The first patient presented a picture characterized by diarrhoea, palpitations and effort dyspnea demonstrating heart involvement suggestive of eosinophilic infiltration on echocardiographic study. The second patient presented dyspneic night cough and generalized pruritus in addition to anemia and thrombocytopenia. The administration of alpha interferon (3 x 10(6) U/3 days a week and 3 x 10(6) U/day, respectively) was followed in both cases by the rapid disappearance of the symptomatology, normalization of the hematologic parameters and a reduction of the eosinophilia to values of less than 1 x 10(9)/l. This response has maintained over the two years of control in which the patients have received alpha interferon as a maintenance treatment.

Abnormal chromatin clumping syndrome (ACCS) is a rare granulocyte disease halfway between Ph'-negative chronic myelogenous leukaemia (CML) and the myelodysplastic syndromes. A new case of such syndrome is presented, the patient being a 65 year-old man with anaemia, thrombocytopenia and myelaemic leucocytosis with abnormal condensation of the neutrophils' chromatin. Marked granulocytic hyperplasia was present in the bone marrow with strikingly dysplastic features, along with a decrease of the remaining blood cell lines. Irregular nuclear outlines and heterochromatin clumps separated by euchromatin bands were seen in the more mature granulocytes upon ultrastructural studies. No cytogenetic abnormalities were found in the bone marrow, and no ber-abl gene rearrangement was present in white cells from peripheral blood. The clinical course was marked by persistence of the anaemia and increasing leucocytosis which required 6-mercaptopurine treatment. Acute myeloblastic leukaemia occurred one year later and the patient died shortly after. It was concluded, from this case and others reported in the literature, that, with regard to its clinical course and proliferative character, ACCS is closer to Ph'-negative, bcr-abl negative CML rather than to the myelodysplastic syndromes.

Purpose: To analyse the initial and evolutive characteristics of the patients with low-grade lymphoma and long survival (> or = 12 years)., Material and Methods: The initial data (clinical, laboratory, histologic type, stage) and the clinical course (response to therapy, response duration) of 52 patients with low-grade lymphoma diagnosed before 1980 were analysed. The statistical study was carried out by means of the chi-square test with Yates' correction and Student's t; the actuarial survival was estimated, and the duration of remission was assessed by the Kaplan and Meier method. Curves were compared by the log rank method., Results: The 12-year survival as a whole was 32% (CI: 95%: 18.5%-45.5%). Of the 52 patients, 13 (25%) survived over 12 years. Nine were men and 4 women, with median age of 41 years (range: 22-68 years). Three cases had lymphocytic lymphoma and 10 had follicular lymphoma; 2 were in stage I-II and 11 in stage III-IV. Those patients who would become long-survivors had at diagnosis lesser frequency of spread lymph-node regional involvement (> or = 3 sites): 46% vs 81.8%, p = 0.04, as well as lesser percentage of bone-marrow involvement (38.4% vs 78.1%, p = 0.023). They were also better responders to treatment (CR+PR = 100% vs 71%, p = 0.013) and with longer duration (actuarial median duration of response 8 years vs 1.5 years, p = 0.005)., Conclusions: According to the present analysis, low-grade lymphoma patients who have at diagnosis low tumoral involvement (not too extensive lymph-node involvement, disease-free bone-marrow) and show good response to treatment have a better prognosis and may hope for a longer survival.

Background: To evaluate the efficacy of i.v. amphotericin B (AmB) as prophylaxis of deep mycosis (DM) in allogenic bone marrow transplantation (BMT)., Methods: From July 1991 to May 1993, 45 consecutive patients treated by allogenic BMT with no previous history of systemic mycosis and with normal renal function were administered prophylactic AmB at a dose of 0.5 mg/kg/48 h from day + 1 BMT until hemoperipheral recovery (group A). These were compared with an historic control group made up of 45 consecutive patients submitted to BMT from January 1990 to June 1991 who did not receive prophylactic AmB (group B). During the neutropenic phase all the patients remained in isolation units with laminar flow of filtered air and were administered oral non absorbable antibiotic therapy and diet of low bacterial content. The incidence of DM and the dose of AmB administered during the first 120 days post BMT were evaluated., Results: In the first 30 days following BMT 3 (7%) cases of DM were observed in group A and 3 (7%) in group B. Four (9%) additional cases were found from days 30 to 120 in group A and 3 (7%) in group B. In 3 (7%) patients of the group which received prophylaxis and in 4 (9%) of the control group Candida spp. was isolated. In 3 (7%) patients from group A and 1 (2%) patient from group B the infection was due to Aspergillus. Although the patients from group A received therapeutic AmB less frequently (78% vs 91%) and later (13 [SD +/- 5.9] vs 9.2 [SD +/- 4.6] days) than those of group B (p < 0.002) the mean dose of AmB per patient treated was similar in both groups (11.3 [SD +/- 8.8] vs 11.8 [SD +/- 7] mg/kg)., Conclusions: The prophylactic use of systemic amphotericin during the neutropenic phase of bone marrow transplantation does not reduce either the incidence of deep mycosis or the mean dose of amphotericin administered.

Paraplegia is a fearful and not uncommon complication of aortic clamping in surgical procedures involving thoracic and abdominal aorta. We report a case of transient spinal cord ischemia during the early postoperative period of aortobifemoral bypass in a 69-year-old male with arteriosclerosis obliterans, hypertension, type II diabetes mellitus and COLD. The anesthetic procedure was combined (peridural + intubation and mechanical ventilation + isofluorane). Two hypotensive episodes of about 80 mmHg developed, one after induction and another in the Reanimation area. The first one had a short duration, whereas the second one required the administration of colloids, crystalloids and blood. The infrarenal aortic clamping time was 35 minutes. In the early postoperative period the patient had clinical features consistent with spinal ischemia, which progressively recovered. To prevent spinal ischemia during surgery a shorter duration than 30 minutes of aortic clamping, a higher distal perfusion pressures higher than 60 mmHg during clamping, and the attempt to exclude the least possible number of intercostal and/or lumbar vessels are recommended. Drugs (corticosteroids, naloxone) and hypothermia can be useful.

Escape rhythms characteristics after successful catheter ablation of the atrioventricular junction and intentional complete heart block were compared with those due to others etiologies as well as the advantage of the posterior implantation of a rate responsive pacemaker (VVIR). In 22 patients, 9 men and 13 women, with permanent AV block after fulguration and follow-up periods from 4 to 58 months, escape rhythms data studied during the procedure and at short and long-term periods, showed the following characteristics: 1) Classical parameters of valuation are not as a whole useful to evaluate subsidiary escape rhythms after electrical fulguration of the atrioventricular junction system. 2) The QRS morphology of the escape rhythm exhibited frequently right or left bundle branch block and specially the former. 3) Spontaneous variability of the escape rhythm recovery time makes this parameter unreliable. 4) Electrophysiological parameters previous to the shock and energy delivery do not predict the origin and characteristics of the subsidiary escape rhythms. 5) Active ventricular arrhythmias appearing immediately postshock are unrelated with any subsequent complication. 6) Permanent pacing increases pacemaker dependency at least temporarily. In 15 of 22 patients, a rate responsive multiprogrammable pacemaker controlled by QT interval was implanted. Cardiac performance and life-quality was improved in these patients due to the addition of several factors: 1) Control of the tachyarrhythmias. 2) Elimination of antiarrhythmic drugs and their side-effects on ventricular function. 3) Self-regulation of cardiac rate through permanent stimulation with a QT sensitive rate responsive pacemaker.

The authors present three cases of perineal, testicle ectopia, two of which were diagnosed and treated in the Urology Department of the "La Fe" Hospital. The other observation was diagnosed and treated by one of us privately. The treatment was surgical in all the cases and good results were obtained in them all. They discuss the etiopathogeny, clinical characteristics and the uselessness of hormone treatment.

On the occasion of a recent observation in our Centre of a case of congenital renal arteriovenous fistula, we make a review of the literature and give a detailed description of the clinical case presented. We stress the difficulty in differentiating between congenital and acquired fistulae and explain the course of therapy currently used.