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      Effusive-Constrictive Pericarditis Secondary to Primary Pericardial Lymphoma: A Case Report

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          Abstract

          Few cases of primary cardiac lymphoma (PCL) are found in the literature. We report the case of an 85 year-old male who presented with cardiac tamponade and effusive-constrictive pericarditis secondary to primary cardiac lymphoma involving only the pericardium. There have been no prior published cases with these rare scenarios.

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          Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns.

          S Cho, Adam M. Petrich, Henny Billett (2011)
          Primary cardiac lymphoma (PCL) represents a rare subset of non-Hodgkin lymphoma, characterized by poor outcomes. The authors aimed to construct a framework of known clinical presentations, diagnostic features, disease complications, treatment, and outcomes to improve prognostication. Individual patient data were obtained from defined cases of PCL (1949-2009) and systematically analyzed. The authors report results of a review of 197 cases of PCL, with half of all cases reported since 1995. Survival was affected by 4 factors: immune status, left ventricular involvement, presence of extra-cardiac disease, and arrhythmia. Median overall survival (OS) for immunocompromised and immunocompetent was 3.5 months (m) and not reached, respectively (HR 0.29, 95% CI, 0.13-0.68; P = .004). LV involvement was uncommon (26%) and associated with an OS of only 1 m, whereas patients free of LV involvement had a median OS of 22 m (HR 0.28, 95% CI, 0.12-0.64; P = .002). Patients with extracardiac disease had shorter median OS compared with those without (6 m vs 22 m, HR 0.49, 95% CI, 0.26-0.91; P = .02). Those patients with an arrhythmia of any type had a median OS that was not reached (n = 55), whereas those without rhythm disturbances (n = 41) had median OS of 6 m (HR 0.51, 95% CI, 0.29-0.91; P = .024). Overall response rate to therapy was 84%, with long-term OS over 40%. The current study presents the largest analysis of PCL to date. The data demonstrate that PCL is now more frequently diagnosed premortem and appears to have reasonable response rates. Lack of LV involvement and the presence of arrhythmias are associated with improved survival. Copyright © 2010 American Cancer Society.
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            Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.

            G Ceresoli, M Ponzoni, A Ferreri … (1997)
            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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              Effusive-constrictive pericarditis.

              Juan R Angel, Jaume Sauleda, Gaietà Permanyer-Miralda … (2004)
              Effusive-constrictive pericarditis is an uncommon pericardial syndrome characterized by concomitant tamponade, caused by tense pericardial effusion, and constriction, caused by the visceral pericardium. We conducted a prospective study of its clinical evolution and management. From 1986 through 2001, all patients with effusive-constrictive pericarditis were prospectively evaluated. Combined pericardiocentesis and cardiac catheterization were performed in all patients, and pericardiectomy was performed in those with persistent constriction. Follow-up ranged from 1 month to 15 years (median, 7 years). A total of 1184 patients with pericarditis were evaluated, 218 of whom had tamponade. Of these 218, 190 underwent combined pericardiocentesis and catheterization. Fifteen of these patients had effusive-constrictive pericarditis and were included in the study. All patients presented with clinical tamponade; however, concomitant constriction was recognized in only seven patients. At catheterization, all patients had elevated intrapericardial pressure (median, 12 mm Hg; interquartile range, 7 to 18) and elevated right atrial and end-diastolic right and left ventricular pressures. After pericardiocentesis, the intrapericardial pressure decreased (median value, -5 mm Hg; interquartile range, -5 to 0), whereas right atrial and end-diastolic right and left ventricular pressures, although slightly reduced, remained elevated, with a dip-plateau morphology. The causes were diverse, and death was mainly related to the underlying disease. Pericardiectomy was required in seven patients, all of whom had involvement of the visceral pericardium. Three patients had spontaneous resolution. Effusive-constrictive pericarditis is an uncommon pericardial syndrome that may be missed in some patients who present with tamponade. Although evolution to persistent constriction is frequent, idiopathic cases may resolve spontaneously. In our opinion, extensive epicardiectomy is the procedure of choice in patients requiring surgery. Copyright 2004 Massachusetts Medical Society
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                Author and article information

                Journal
                Journal ID (nlm-ta): World J Oncol
                Journal ID (iso-abbrev): World J Oncol
                Journal ID (publisher-id): Elmer Press
                Title: World Journal of Oncology
                Publisher: Elmer Press
                ISSN (Print): 1920-4531
                ISSN (Electronic): 1920-454X
                Publication date (Print): April 2012
                Publication date (Electronic): 23 April 2012
                Volume: 3
                Issue: 2
                Pages: 87-90
                Affiliations
                [a ]Graduate Medical Education - Cardiovascular Medicine Fellowship, Kettering Medical Center, USA
                [b ]Internal Medicine Residency Program, Kettering Medical Center, USA
                [c ]Dayton Cancer Center, Kettering Health Network , USA
                Author notes
                [d ]Corresponding author: Mauricio Anaya-Cisneros, 3535 Southern Boulevard, Dayton, OH 45429, USA. Email: mauricioanaya1@ 123456hotmail.com
                Article
                DOI: 10.4021/wjon449w
                PMC ID: 5649895
                PubMed ID: 29147286
                SO-VID: e1e5233e-507e-456f-85c3-0603c4f4f167
                Copyright © Copyright 2012, Anaya-Cisneros et al.
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date accepted : 29 December 2011
                Categories
                Subject: Case Report

                Keywords: pericardial lymphoma,extranodal nhl,cardiac tamponade,effusive-constrictive pericarditis,rituximab,liver failure
                Data availability:
                Keywords: pericardial lymphoma, extranodal nhl, cardiac tamponade, effusive-constrictive pericarditis, rituximab, liver failure

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