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R Forés

Publications and source records attributed to R Forés.

25 records · Page 2Linked to original sources

Flow cytometric analysis of decay-accelerating factor (CD55) on neutrophils from aplastic anaemia patients.

Using a flow cytometric analysis, CD55 (decay-accelerating factor), CD59 and CD58 have been measured on neutrophils from 12 aplastic anaemia (AA) patients who were long-term survivors after immunosuppressive therapy (IS), 17 healthy individuals, four patients with PNH, and six patients with other haematological disorders. The neutrophils from normal control patients and the six patients with other haematological disorders showed 98 +/- 2% (mean +/- SD) positive granulocytes for CD55. Corresponding values were low (12%, 26%, 51% and 58%) on the primarily PNH patients. Among the 12 AA patients examined, seven had normal and five low values (59% in two, 70%, 71% and 82%). Among the five AA patients who showed CD55 neutrophil deficiency, four had showed an incomplete response after the initial IS treatment and the other relapsed following an initial haematological complete response; three cases had a positive Ham's test and two were negative. Our data suggest that the development of PNH clones is a frequent finding in AA long-term survivors, mainly in those who had shown an incomplete response following IS. Neutrophil CD55 expression analysis by flow cytometry could be useful to detect clonal evolution in these patients.

Adult↗

[Efficacy of various treatments in the management of idiopathic thrombocytopenic purpura in the adult].

PURPOSE: To evaluate the ITP treatment modalities and their results in a series of 30 adult patients. PATIENTS AND METHODS: 30 patients, age 15-74, were diagnosed of ITP in our institution between 1988 and December 1991. We defined the ITP as: mild (platelets > or = 50 x 10(9)/L and < or = 120 x 10(9)/L), moderate (platelets 25-50 x 10(9)/L) and severe (platelets < or = 25 x 10(9)/L). Treatment was initiated when the platelet count reached a level < 50 x 10(9)/L. Initial therapy was oral prednisone or methyl-prednisolone in all patients. In 12 unresponsive patients the treatment included splenectomy. Patients who fail steroid therapy and splenectomy received additional lines of therapy, that included: vincristine (n = 2), vinblastine (n = 1), danazol (n = 5), anti D immunoglobulin (n = 6), methotrexate (n = 2), intravenous immunoglobulin (n = 3). RESULTS: Complete remission was obtained in 19 patients with a follow-up of 3 to 55 months (median, 30 months). Partial remission was achieved in 4 patients, that was sustained for 9 to 39 months (median, 22.5 months) after initial therapy. One unresponsive patient died of infection. Six mild ITP patients did not require therapy with a follow-up of 14 to 56 months (median, 33.1 months). CONCLUSION: In adult patients with mild ITP, therapy is not required. Steroid therapy remains the initial therapeutic choice for moderate and severe ITP. In unresponsive patients useful alternative choices are splenectomy, danazol, anti D immunoglobulin and methotrexate.

Adolescent↗

[Treatment with fludarabine of chronic refractory lymphoid leukemia].

PURPOSE: New antimetabolic drugs, purine-analogous, have been introduced in the treatment of advanced or refractory cases of chronic lymphocytic leukaemia (CLL), fludarabine (FLU) being one of such drugs. The results attained with FLU in 6 cases of refractory CLL are reported. PATIENTS AND METHODS: The efficacy and toxicity of FLU was evaluated in 6 patients (median age 63 years) between March 1993 and March 1994. Five patients were in stage III-IV and one in stage II of the Rai's system, and they were refractory to the usual treatment. The dosis used here was 30 mg/sq m/day, for 5 days every 4 weeks, up to a total of 6 therapeutic courses. The response was assessed after 3 and 6 courses. Anti-infectious prophylaxis was made with co-trimoxazole, monthly benzathine penicillin G and isoniazid in Mantoux-positive patients. The response criteria were those given by the NIC Working Group for CLL. RESULTS: The patients received 2 to 6 courses. None of them attained complete remission: 1 had nodular remission, 2 had partial remission, 2 responded partially although not reaching criteria for partial remission (1 died of pancytopenia), and 1 had disease progression and died after the second course. Fast and important reduction of the lymphocyte count as well as the CD4 lymphocytes was present in all cases. Bone-marrow infiltration decreased strikingly in 2 cases and the platelet count improved in 3 cases and worsened in 2 others. The following toxicity was recorded: 2 patients had nausea, 5 had neutropenia (below 500 x 10(9)/L in 3 instances) and 4 had thrombocytopenia (lower than 40 x 10(9)/L in one case). In 26 therapeutic courses there were 10 febrile episodes (1 for pneumonia, 1 for gastroenteritis and 8 without any septic foci); 1 patient developed pulmonary tuberculosis after completing the treatment and one patient died of posttransfusion graft versus host disease after splenectomy once she had completes six courses. CONCLUSION: This experience confirms the efficacy of FLU in the treatment of refractory B-CLL patients and is in agreement with previous reports as no response is initiated after the 3rd course. The lymphocyte count decreases quickly and strikingly. Depletion of CD4 lymphocytes along with neutropenia and hypogammaglobulinaemia make these patients highly sensitive to all types of infection, chiefly by opportunistic germs, so adequate anti-infectious prophylaxis is of great importance. The two patients with pre-treatment haemoglobin and platelet values lower than 10 g/dL and 40 x 10(9)/L, respectively, were the only ones in need of transfusion. No cumulative myelosuppression was appreciated.

Aged↗

De novo smoldering paroxysmal nocturnal hemoglobinuria: a flow cytometric diagnosis.

An asymptomatic 26-year-old woman with mild macrocytic anemia (11.6 g Hb/dl) was studied. All biochemical parameters, bone marrow histology and cytogenetics were normal. The Ham's and sucrose tests were negative. A flow cytometric analysis revealed that CD55 and CD59 staining was absent in 20% and 21% of the granulocytes, but erythrocytes and CD34-positive bone marrow cells were CD55 and CD59 positive. Seven months after the initial study, the patient suffered an episode of hemoglobinuria, with mild anemia, moderate thrombocytopenia and a weak positive sucrose lysis test. A new flow cytometric analysis disclosed an increased percentage of CD55 and CD59 negative granulocytes and a 25% of erythrocytes with an intermediate pattern of fluorescence after CD59 labelling. At fourteen months, a population of CD55-deficient erythrocytes was detected and the Ham's test became positive. The present report is, to our knowledge, the first case of smoldering paroxysmal nocturnal hemoglobinuria in a patient with no previous aplastic anemia, or evident pancytopenia. The diagnosis was established by flow cytometry of peripheral blood granulocytes, with apparently phenotipically normal progenitor cells in an early stage of the disease. Flow cytometry appears to be a useful tool in our knowledge of paroxysmal nocturnal hemoglobinuria evolution.

Adult↗