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      Linfoma cardiaco primario: diagnóstico citológico y tratamiento con respuesta a poliquimioterapia y a autotrasplante de precursores hematopoyéticos. Presentación de un caso y revisión de la literatura Translated title: Primary cardiac lymphoma: antemortem cytologic diagnosis and treatment with a response to polychemotherapy and the autologous peripheral blood stem cells transplantation. Presentation of one case report and review of the literature

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          Abstract

          El linfoma cardíaco primario (LCP) es un tumor extraordinariamente infrecuente en pacientes inmunocompetentes, de diagnóstico dificil y a menudo tardío, que comporta un pronóstico muy grave y escasas posibilidades terapeúticas. Presentamos un caso de linfoma no Hodgkin cardíaco primario en un paciente de 46 años, inmunocompetente, que debutó con insuficiencia cardíaca y flutter auricular. Se practicaron estudios con ecocardiografía transesofágica (TEE), tomografía computarizada (TC) y resonancia magnética (MR) que detectaron una tumoración intracardíaca localizada a nivel del septo interauricular. El diagnostico se fundamentó en el examen citológico del liquido pleural. Se instauró tratamiento quimioterápico y se sometió al paciente a un autotrasplante de precursores hematopoyéticos. El paciente permanece en remisión 36 meses después del diagnóstico y 29 meses del autotrasplante. Nuestra experiencia indica que un diagnóstico precoz y preciso conjuntamente con un tratamiento antilinfomatoso apropiado y agresivo puede ayudarnos a obtener una supervivencia prolongada en pacientes con LCP.

          Translated abstract

          The primary cardiac lymphoma (PCL) is an extremely infrequent tumor suffered by immunocompetents patients with a difficult diagnosis and slow progress leading to a serious prognosis and few therapeutically possibilities. It's a primary-cardiac non-Hodgkin's lymphoma (NHL) in a patient of 46-year-old, immunocompetent, who started with a congestive heart failure and atrial flutter. Some examinations were carried out such as a transesophageal echocardiography (TEE), a computed tomography (TC) and a magnetic resonance imaging (MRI) and an intracardiac tumor placed in the interauricular septum was detected. The diagnosis was based on a pleural fluid cytological examination. It was decided to follow a chemotherapy treatment and the autologous peripheral blood stem cells transplantation was carried out. The patient remains in full remission thirty-six months after diagnosis and twenty-nine months after the autotransplant. Our clinical experience indicated that an early and accurate diagnosis combined with the appropriate and aggressive antilymphoma therapy can thus help in obtaining a long survival in patients with PCL.

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          Most cited references76

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          Autologous bone marrow transplantation as compared with salvage chemotherapy in relapses of chemotherapy-sensitive non-Hodgkin's lymphoma.

          High-dose chemotherapy followed by autologous bone marrow transplantation is a therapeutic option for patients with chemotherapy-sensitive non-Hodgkin's lymphoma who have relapses. In this report we describe a prospective randomized study of such treatment. A total of 215 patients with relapses of non-Hodgkin's lymphoma were treated between July 1987 and June 1994. All patients received two courses of conventional chemotherapy. The 109 patients who had a response to chemotherapy were randomly assigned to receive four courses of chemotherapy plus radiotherapy (54 patients) or radiotherapy plus intensive chemotherapy and autologous bone marrow transplantation (55 patients). The overall rate of response to conventional chemotherapy was 58 percent; among patients with relapses after chemotherapy, the response rate was 64 percent, and among those with relapses during chemotherapy, the response rate was 21 percent. There were three deaths from toxic effects among the patients in the transplantation group, and none among those in the group receiving chemotherapy without transplantation. The two groups did not differ in terms of prognostic factors. The median follow-up time was 63 months. The response rate was 84 percent after bone marrow transplantation and 44 percent after chemotherapy without transplantation. At five years, the rate of event-free survival was 46 percent in the transplantation group and 12 percent in the group receiving chemotherapy without transplantation (P = 0.001), and the rate of overall survival was 53 and 32 percent, respectively (P = 0.038). As compared with conventional chemotherapy, treatment with high-dose chemotherapy and autologous bone marrow transplantation increases event-free and overall survival in patients with chemotherapy-sensitive non-Hodgkin's lymphoma in relapse.
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            Primary sarcomas of the heart.

            Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.
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              Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.

              Primary cardiac lymphoma (PCL) is extremely rare in immunocompetent patients. Different definition criteria have been employed in published series. Prognosis is poor due to diagnostic delay and relevance of the site of disease. Two cases observed at the study institution are reported, with a review of 48 cases published in the literature from 1980 to 1996. Only patients with lymphoma confined to the heart and/or pericardium and those with a single and asymptomatic extracardiac site were considered for analysis. Eight patients had minimal extracardiac disease. The most common presentation was unresponsive heart failure. Electrocardiography findings were not specific. PCL usually arose in the right chambers as a mass, with or without pericardial effusion (> 80%). Chest X-rays, transthoracic echocardiography, and computed tomography scans are standard in diagnostic workup, but transesophageal echocardiography (TEE) and magnetic resonance imaging (MRI) showed a sensitivity > 90%. Cytology of pericardial effusion was diagnostic in 67% of cases. Thoracotomy was diagnostic in all cases, whereas less invasive procedures had high false-negative rates. Gross resection has no role. Early anthracycline-containing chemotherapy appears to improve survival, whereas the role of radiotherapy has not yet been defined. The diagnosis of PCL should be considered in patients with a cardiac mass and/or unexplained refractory pericardial effusion. Adequate diagnostic workup, including TEE and MRI, allows confirmation of the early suspicion of PCL. In the absence of a diagnostic cytology, an open biopsy may be indicated to avoid treatment delay. There is no evidence that PCL should be treated differently from other bulky aggressive lymphomas arising at other anatomic sites.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                ami
                Anales de Medicina Interna
                An. Med. Interna (Madrid)
                Arán Ediciones, S. L. (, , Spain )
                0212-7199
                June 2002
                : 19
                : 6
                : 45-51
                Affiliations
                [02] orgnameHospital Universitari de Girona. Dr. Josep Trueta orgdiv1Servicio Hematología
                [01] orgnameHospital Universitari de Girona. Dr. Josep Trueta orgdiv1Servicio Medicina Interna
                [03] Girona orgnameHospital Universitari de Girona. Dr. Josep Trueta orgdiv1Servicio Cardiología
                Article
                S0212-71992002000600008
                10.4321/s0212-71992002000600008
                f31731ef-e43f-4734-aa61-62e3d3514f21

                This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 International License.

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                SciELO Spain


                Linfoma cardíaco primario,Diagnóstico,Pronóstico,Tratamiento,Primary cardiac lymphoma,Diagnosis,Prognosis,Treatment

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