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Biomedical subjects

J Juncà

Publications and source records attributed to J Juncà.

At least 19 recordsLinked to original sources

CXCR4 and SDF-1 expression in B-cell chronic lymphocytic leukemia and stage of the disease.

The pathogenesis of B-cell chronic lymphocytic leukemia (B-CLL) has been linked to an overexpression of the chemokine receptor CXCR4 and increased in vitro functional response to its natural ligand CXCL12 (SDF-1). The CXCR4/SDF-1 system appears to be important for tissue localization and increased survival of B-CLL cells. The aim of our study was to examine if CXCR4 expression and SDF-1 blood levels were correlated to clinical and pathological stage of B-CLL. Flow cytometry and enzyme-linked immunosorbent assay (ELISA) techniques were used to determine CXCR4 expression and SDF-1 plasma levels, respectively, in a cohort of 51 patients diagnosed with B-CLL to correlate these measurements with several parameters that define the clinical stage of the disease. We confirmed that CXCR4 was consistently expressed on circulating B-CLL cells with a fluorescence intensity that was five-fold greater than in cells from healthy volunteers. There was a correlation between CXCR4 expression and leukocyte count ( r: 0.55, p<0.01), and CD19(+)/CD5(+ )cells ( r: 0.63, p<0.01). Interestingly, the group of B-CLL patients showed lower SDF-1 plasma levels compared to the control group. However, there was no correlation between CXCR4 or SDF-1 expression and the clinical stage of disease or the pattern of bone marrow infiltration. The results obtained suggest that other factors, and not only alteration in the SDF-1/CXCR4 chemokine system, must account for marrow infiltration of neoplastic cells observed in B-CLL and that CXCR4 could be involved in other features that exhibit malignant B cells, such as increased survival, rather than in their homing or migration to the bone marrow.

Adult↗

Homocysteine and lipid peroxidation in haemodialysis: role of folinic acid and vitamin E.

BACKGROUND: Cardiovascular diseases are the leading cause of death in haemodialysis patients. Hyperhomocysteinaemia is an independent risk factor. Basic research has provided strong evidence that oxidation of low-density lipoprotein (LDL) plays an important role in the pathogenesis of atherosclerosis. Oxidative stress, lipid metabolism alterations, and hyperhomocysteinaemia observed in haemodialysis patients could induce increases in LDL oxidation. This study was designed to determine the effect of folinic acid on hyperhomocysteinaemia and to assess the antioxidant efficacy of folinic acid. The antioxidant effect of folinic acid was compared with that of vitamin E. METHODS: Sixteen stable patients (11 men, five women; mean age 54.3+/-6.32 years) on standard haemodialysis received 400 mg of vitamin E, orally, at the end of each haemodialysis session for 3 months. After a 1-month wash-out, they received 10 mg of folinic acid, intravenously, at the end of each haemodialysis session for an additional 3 months. Blood samples were drawn in the morning after an overnight fast and before dialysis. Plasma vitamin E was analysed by high-pressure liquid chromatography. Malondialdehyde (MDA) was determined using a fluorimetric method and plasma copper oxidized anti-LDL antibodies (Ab-LDLox) were measured with an ELISA method using native LDL and oxLDL as antigens. Plasma homocysteine was determined by an FPIA method. RESULTS: Folinic acid supplements significantly reduced hyperhomocysteinaemia (-44%), MDA concentrations (-40%), and IgG-LDLox titres (-13%). CONCLUSIONS: Treatment with folinic acid lowers plasma homocysteine levels and, like vitamin E, affords antioxidant protection, which prevents lipid peroxidation. This lowering of lipid peroxidation may reduce the risk of atherosclerosis and prevent or delay cardiovascular complications in HD patients.

Antioxidants↗

The relationship between idiopathic thrombocytopenic purpura and pernicious anaemia.

Some reports on the simultaneous presence of chronic idiopathic thrombocytopenic purpura (ITP) and pernicious anaemia (PA) may be found in the literature. However, little is known about the coexistence of these autoimmune disorders. For this reason, we studied the prevalence of PA in a series of patients with a diagnosis of chronic ITP by means of the analysis of the concentration of the most sensitive marker of type A atrophic (autoimmune) gastritis, serum pepsinogen I. Serum pepsinogen I was low in 20.3% of the 133 patients studied. Gastrin was elevated in 15. 2% of patients, but the coexistence of both abnormalities was rather low (7.6% of patients). However, the progressive decrease in serum cobalamin as biochemical abnormalities related with atrophic gastritis appeared was noticeable. The time to progression to frank PA from type A atrophic gastritis may span some years.

Adult↗

Erythropoietin plus granulocyte colony-stimulating factor in the treatment of myelodysplastic syndromes. Identification of a subgroup of responders. The Spanish Erythropathology Group.

BACKGROUND AND OBJECTIVE: Anemia leading to transfusion is probably the most important problem in patients with myelodysplastic syndromes (MDS). Human recombinant erythropoietin (rHuEpo) and granulocyte colony-stimulating factor (G-CSF) have been used to treat patients with anemia of MDS, but fewer than 50% respond. The aim of this work was to evaluate the benefit of rHuEpo +/- G-CSF treatment and to isolate the response predictive variables in a group of selected patients with MDS. DESIGN AND METHODS: A non-randomized multicenter trial was carried out in 32 patients with MDS. The inclusion criteria were age >= 18 years, refractory anemia (RA) or refractory anemia with ringed sideroblasts, Hb <= 100 g/L or receiving transfusions and serum erythropoietin <= 250 U/L. These patients were treated with subcutaneous rHuEpo (300 U/kg) three times a week for 8 weeks. In the case of partial response (PR) or no response (NR) subcutaneosly administered G-CSF (1 microg/kg) three times a week was added to the rHuEpo for 8 more weeks. If the patient achieved complete response (CR) or PR in the second phase, he was included in a follow-up phase of 24 weeks in which the dose of growth factors was tapered down. Several variables, including the score published by the Scandinavian-American group, were used as possible predictive variables. RESULTS: An erythroid response was observed in 16 patients (50%); in 12 it was a CR and in 4 it was a PR. During the period of rHuEpo administration, 7 CR and 4 PR (34.4%) were documented. Of the 14 patients in whom G-CSF was added to rHuEpo, 7 (50%) responded (3 CR and 4 PR). No major side-effects associated with growth factors were observed. The multivariate analysis showed that of the different variables evaluated only the Scandinavian-American response score was significant with a relative probability of response of 11.8 (95% confident intervals: 2.5-53) when this score was > +1 (77% of cases responded). In contrast, when this score was <= 1 only 15 % of the cases responded. INTERPRETATION AND CONCLUSIONS: Use of the Scandinavian-American response score is to be recommended in a patient-oriented approach to treating MDS cases with the Epo and G-CSF. Treatment with rHuEpo and G-CSF is safe, its main drawback being its cost. However, a long-term study evaluating the regimen's cost-benefit ratio is warranted.

Adult↗

The usefulness of the serum transferrin receptor in detecting iron deficiency in the anemia of chronic disorders.

BACKGROUND AND OBJECTIVE: Recent studies have shown that the serum transferrin receptor (sTfR) is a sensitive, quantitative measurement of tissue iron deficiency. The objective of the study was to evaluate the diagnostic efficiency of some laboratory tests, including sTfR measurements, in the diagnosis of iron depletion in patients with anemia of chronic disorders. DESIGN AND METHODS: The patient population consisted of 37 anemic patients: 10 hypoferritinemic patients (serum ferritin < 25 micrograms/L), and 27 anemic in-patients with hyperferritinemia (serum ferritin > 200 micrograms/L) and clinical/analytical criteria of anemia of chronic disorders, who were submitted to a bone marrow aspirate with iron stain. The sensitivity and specificity of serum TfR was evaluated according to the results of bone marrow iron status. Statistical analysis employed Student's t-test, one way analysis of variance and a logistic regression model using the Wald test. RESULTS: Serum TfR was high in all the patients with hypoferritinemic anemia. In 12 patients with low bone marrow iron, the mean sTfR was 5.63 mg/L. In 6 of these 12 patients the sTfR was normal. On the other hand, sTfR was high in 4/15 patients with normal or increased iron stores. On multivariate analysis the most sensitive predictor of true iron deficiency was MCH (mean corpuscular hemoglobin). No other variables remained independently significant, including sTfR, after the inclusion of MCH in this model. INTERPRETATION AND CONCLUSIONS: In our opinion, the iron status of patients with anemia of chronic diseases can not be accurately assessed by sTfR, as its sensitivity and specificity are low. In these patients, the gold standard for iron stores evaluation continues to be bone marrow aspirate and Perls stain.

Adult↗

[Acute myelomonocytic leukemia with eosinophilia and inversion of chromosome 16. Study of 6 cases].

The purpose of this study has been to refer the main clinico-biologic characteristics, the evolution and the response to therapy in 6 patients with acute myelomonocytic leukemia with eosinophilia and inversion of chromosome 16 (AML4Eo inv[16]) belonging to a series of 92 patients with acute myeloblastic leukemia diagnosed in a single hospital between 1987 and 1995. The main clinical manifestations were anemic syndrome and hemorrhage. Anemia and thrombocytopenia were present in all cases, high white blood cell count in 4, monocytosis in 5 and eosinophilia in one. Bone marrow aspirate showed myeloid and monocytic blast infiltration (43-62%), eosinophilia (5-19%) and atypical monocytic precursors (6-18%). Induction therapy consisted in one or two cycles of daunorubicin (or idarubicin), cytosine arabinoside and etoposide, followed by two cycles of consolidation treatment, the first with mitoxanthrone and cytosine arabinoside and the second with amsacrine and cytosine arabinoside. One patient died in the induction phase, while complete remission was obtained in the remaining 5. One patient died during the consolidation therapy. Allogeneic bone marrow transplant (BMT) was performed to one patient and autologous BMT to another. The first patient remains in complete remission (CR) at 68 months from diagnosis, and the second relapsed 12 months after BMT. Another patient relapsed at 13 months from diagnosis and the remaining persists in CR 13 months from diagnosis. Actuarial probabilities of CR duration and survival were 50% at 5 years. The clinico-biologic characteristics and the response to therapy of patients with AML4Eo inv(16) are similar to those referred in other series. There is a high probability of CR attainment and, probably, the relapse rate is lower than that of other subtypes of AML.

Adolescent↗

Electron microscopic identification of parvovirus virions in erythroid and granulocytic-line cells in a patient with human parvovirus B19 induced pancytopenia.

In this short report we describe a patient with human parvovirus B19 (HPV B19)-induced transient pancytopenia. Parvovirus virions were seen by electron microscopy in both erythroid and granulocytic precursors. Erythroid cells are not the only targets in these cases. We draw attention to this disorder so that physicians involved with hematological disorders and transplantation be more aware of this infection.

Adult↗