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BACKGROUND AND OBJECTIVE MYELOFIBROSIS: is an infrequent chronic myeloproliferative neoplasm. We aimed to describe the clinico-biological characteristics, treatment, and evolutive course of myelofibrosis patients in Spain., Material and Methods: A total of 1,000 patients from the Spanish Registry of Myelofibrosis diagnosed with primary (n=641) or secondary (n=359) myelofibrosis were analysed., Results: Median age was 68 years. The frequency of constitutional symptoms, moderate to severe anaemia (Hb<10g/dL), and symptomatic splenomegaly was 35%, 36%, and 17%, respectively. The rate of thrombosis and haemorrhage was 1.96 and 1.6 events per 100 patient-years, respectively. The cumulative incidence of leukaemia at 10 years was 15%. The most frequent therapies for the anaemia were the erythropoiesis stimulating agents and danazol. From 2010, a progressive increase in the use of ruxolitinib was noticed. A total of 7.5% of patients were transplanted. During the observation period, 42% of patients died mainly due to the clinical deterioration caused by myelofibrosis or leukaemic transformation. The median survival of the series was 5.7 years. Four different risk categories were identified by the IPSS: median survival was not reached in the low risk group and was 8.8 years, 5.3 years, and 2.8 years in the intermediate-1, intermediate-2, and high-risk groups, respectively., Conclusions: Myelofibrosis is a disabling condition mainly affecting elderly people. Its treatment is mostly driven by symptom control. Despite its clinical heterogeneity, several prognostic models are useful to select candidates for transplantation., (Copyright © 2019 Elsevier España, S.L.U. All rights reserved.)

Background and Objective: To examine the current status of safety practices for medication-use systems in Spanish hospitals and to identify major areas of risk., Material and Method: Those hospitals that completed the "Medication use-system safety self-assessment for hospitals" between June 1 and July 15, 2007, were included in the study. The survey contained 232 items for evaluation grouped into 20 core characteristics., Results: A total of 105 hospitals from the 17 autonomous communities in Spain participated in the study. The average aggregate score for the survey of all the participating hospitals was 612.7 (39.7% of the maximum possible score) and there were no differences found with regard to number of beds, training activity or type of hospital. When core characteristics were analyzed, there were 3 criteria with the lowest values (< 25%), associated with professional training, skills, and the establishment of a system for reporting errors. Another 9 criteria, with percentages between 25% and 50%, reflected practices related to: access to information regarding patients and medications; communication of medication orders; prevention of errors due to naming, labeling, and packaging problems; standardization of medication delivery devices; restriction of medications in patient care units; and safety culture and double-checking procedures., Conclusions: Many opportunities for improvement have been identified, particularly in areas related to training, risk management, incorporating new technologies and patient participation. The information obtained may prove useful for prioritizing practices when establishing patient safety strategies, and as a baseline for successfully monitoring the effectiveness of the initiatives and programs consequently set into motion.

Background: Patients with non-Hodgkin's lymphoma (NHL) have increased serum levels of soluble interleukin-2 receptor (sCD25). In this study the authors investigate: a) the value of sCD25, compared to other serum markers, as tumor marker, and b) the relationship of the sCD25 with the response to therapy and prognosis., Patients and Methods: Serum interleukin-2 receptor (sCD25) levels were measured at diagnosis in 63 patients with NHL (low-grade lymphoma 30 and high-grade lymphoma 33)., Results: High levels of sCD25 were found in these patients compared to a control group (median 1,757 U/ml vs 385 U/ml; p < 0.0001). Significant differences were also found between the high-grade group and the low-grade group, as a whole and within the same Ann Arbor stage. sCD25 showed a correlation coefficient higher than other serum parameters (albumin, LDH, beta 2-microglobulin, uric acid, C-reactive protein) with Ann Arbor stage and with the number of involved lymph nodes or extralymphatic organs. In the high-grade NHL, the median of sCD25 (3,000 U/ml) separates patients with differences in the overall survival (p = 0.0138) and in percentage of complete remisions (p = 0.0079). All the patients with sCD25 < or = 3,000 U/ml reached the remision. The association sCD25 > 3,000 U/ml and albumin < 3.5 g/dl selected to 5 out of 6 patients who failed induction chemotherapy, and only 2 out of 22 who reached the remision., Conclusions: The sCD25 is the best serum factor for estimating tumor burden in NHL. sCD25 level isolates or associated with albumin provides prognostic information.

Background: The most potent stimulator for the hepatic synthesis of C-reactive protein is the interleukin-6. Also interleukin-6 is endowed with thrombopoietic activity, and its seric levels increases in most of secondary thrombocytosis whereas they remain normal in chronic myeloproliferative diseases or primary thrombocytosis. The aims of the study were verify the ability of quantitation of serum C-reactive protein in the differential diagnosis of primary thrombocytosis., Methods: Serum samples from 89 patients with thrombocytosis (> 400 x 10(9)/1) and 54 normal controls were assayed for C-reactive protein. Patients with thrombocytosis were classified in primary thrombocytosis with 27 patients (chronic myeloproliferative disease with thrombocytosis) and secondary thrombocytosis (62 cases)., Results: The mean C-reactive protein serum levels observed in the 27 patients with primary thrombocytosis were 13 +/- 10 mg/l, superior to normal controls (7 +/- 5 mg/l; p < 0.01). In the secondary thrombocytosis group, C-reactive protein serum levels reached a mean value of 59 +/- 34 mg/l, clearly superior to control group and the primary thrombocytosis group (p < 0.0001). No patients in primary thrombocytosis group reached a C-reactive protein value > 40 mg/l, versus 65% of patients in secondary thrombocytosis group. A normal value occurred in 67% cases of primary thrombocytosis group, but also in 17% cases of secondary thrombocytosis group., Conclusions: Quantitation of C-reactive protein could thus prove useful in the differential diagnosis between primary and secondary thrombocytosis.

The case of a patient with the diagnosis of essential thrombocythemia is presented. Following treatment with melphalan during three years the patient presented clinical and radiologic data of pulmonary fibrosis. Thoracotomy with lung biopsy histologically proving fibrosis was performed. The patient developed a true histiocytic lymphoma afterwards. The rarity of pulmonary fibrosis induced by melphalan and the exceptional association of essential thrombocythemia and histiocytic lymphoma is emphasized. The characteristics of the latter disease, diagnostic difficulties and possible treatment are commented upon.

Background: Initially, the aim of the present study was to evaluate the incidence and implicated organisms in the infections in patients receiving a bone marrow transplants (BMT)., Methods: 194 febrile episodes (FE) were evaluated in 115 patients having received a BMT between 1980 and 1987. The analysis was carried out at three different moments: during the period of most marked neutropenia (period I) to the 100th day after BMT (period II) and beyond the 100th day (period III)., Results: The unequivocal confirmation that FE was infective was found in 62% of cases (confirmation was microbiological in 46% and clinical in 16%), while 31% of FE were considered as possible infections and the remaining 7% as doubtful infections. The causative organisms were bacteria (73%), viruses (10%), fungi (8%), and combinations of them (polymicrobial infections) (9%). Gram negative and Gram positive organisms were more common, respectively, in period I and in periods II and III (p = 0.02). Bacteremia was the commonest cause of confirmed infection. The overall mortality rate due to infection was 18%. There was a remarkably high mortality from pneumonia (54%) and a low mortality in patients with sepsis (6%) (p less than 0.0001). The number of FE was lower in patients with autografts than those with allografts (p = 0.08). 33% of the FE in patients with allografts were coincident with acute or chronic graft-versus-host disease, and in two thirds of them infections was confirmed., Conclusions: Infection represents a major complication of BMT. The different antimicrobial treatments used in association with bone marrow grafting allowed us to control most FEs. Pneumonia was the most severe infective localization and the leading cause of death. Mortality rate due to sepsis was small.

Fifteen bone marrow autotransplants (BMAT) in patients with acute myeloblastic leukemia (AML) were performed after the first remission. The mean age was 37 years (range 12 to 60 years). According to the morphological classification FAB, 8 patients had monocytic leukemia (M4, M5) and 7 myeloid leukemia (M1, M2, M3). The mean interval elapsed between the date of complete remission and the BMAT was 3.9 months (range 1 to 5-9 months). In 8 patients this interval was longer than 6 months and in 7 cases it was shorter than 6 months. After achievement of the complete remission all patients underwent certain cycles of intensification before the BMAT. Eight patients received only a cycle whereas 7 patients received more than one cycle (between 2 and 4). The conditioning protocol consisted of cyclophosphamide (CP) (60 mg/kg x 2) and total body radiotherapy (TBR) (10 Gy) in 9 patients; CP and busulfan in five; and CP, cytarabine at high doses and melphalan in one case. Marrow extraction was performed after completion of chemotherapy of intensification. In 5 cases the bone marrow was depleted of leukemic cells by previous in vitro treatment with ASTA-Z. There are at present 8 alive patients. The survival free of illness was 51.8%. Seven patients died: 3 cases because relapse of the leukemia, 3 due to attachment failure of the transplantation, and one patient suffered a viral myocarditis. The survival free of illness was significantly longer in those patients transplanted after 6 months of the complete remission.