A Malignant Squeeze: A Rare Cause of Cardiac Tamponade.
Primary cardiac lymphoma (PCL) is a rare condition that is described as a lymphoma localized to the heart or pericardium with no extracardiac involvement. Although cardiac involvement is seen in about 10-20% of non-Hodgkin's lymphomas, PCL is extremely rare. It comprises merely 0.5% of all lymphomas and 1.3-2% of cardiac malignancies [1, 2]. Clinical presentation typically correlates with the region of the heart involved. It is often a clinical emergency as these patients commonly present with heart failure, cardiac tamponade, or arrhythmia . Herein, we describe a rare case of PCL causing cardiac tamponade in an elderly patient.
2. Case Description
An 85-year-old female with past medical history of recurrent deep venous thrombosis, pulmonary embolism on anticoagulation with a vena cava filter in place, rheumatoid arthritis, hypertension, hyperlipidemia, hypothyroidism, and type 2 diabetes mellitus presented to the emergency department with complaint of exertional dyspnea and chest pain. She denied fever, chills, or lower extremity edema and had no history of malignancy, weight loss, or night sweats. Initial vitals revealed BP of 86/62 mmHg that decreased to 79/60 mmHg with inspiration. Initial pulse was 95 bpm, and respiratory rate was 20. White blood cell count was mildly elevated at 11.5, troponins were normal, and electrocardiogram was unremarkable. D-dimer was elevated at 1290. Patient was sent for CT scan to evaluate for pulmonary embolism, and a moderate pericardial effusion was found (Figure 1). Subsequently, an echocardiogram was done to further delineate the effusion which revealed a large circumferential effusion with mild respiratory variation concerning for impending cardiac tamponade (Figure 2). She was admitted to the intensive care unit and underwent emergent pericardiocentesis. Flow cytometry of the pericardial fluid revealed a population of monoclonal B-cells with significant large cell component (Figure 3). The overall morphologic and immunophenotypic features were consistent with high-grade B-cell lymphoma with t(8; 144 (Figure 4). Bone marrow biopsy demonstrated monotypic B-cells compatible with the diagnosis of large B-cell lymphoma. The patient was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) with an excellent initial response. She was transferred out of the ICU within days and discharged home for outpatient follow-up.
Primary cardiac tumors are known classically to be rare and benign. Cardiac myxoma is the most frequently reported primary cardiac tumor, but hematologic disease, in rare cases, has been known to orginate in the heart . Lymphomas compromise only 0.5-2% of primary cardiac tumors and occur most often in immunocompromised patients, such as those with human immunodeficiency virus (HIV) . The frequencies of other types of non-Hodgkin's lymphoma are detailed in Figure 5 with diffuse large B-cell lymphoma comprising the majority at 30% . PCL does occur in immunocompetent hosts although infrequently, as it did in our patient. In these immunocompetent patients, studies have shown that 80% of PCL are of diffuse B-cell lymphoma . The location of cardiac involvement for PCL is often the right atrium and right ventricle. Clinical presentation can vary depending on the site of involvement of the heart. Most often, an extensive multidisciplinary workup is employed to come up with the correct diagnosis. Imaging modalities such as transthoracic and transesophageal echocardiography, computed tomography (as done in our patient), and magnetic resonance imaging are all utilized in diagnosing this entity. Confirmation of the diagnosis is through cytology of biopsy or pericardial fluid. The combination of chemotherapy and radiation is the treatment of choice. Treatment for B-cell lymphoma is typically an anthracycline-based, such as given in our patient. Our patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Survival has been demonstrated in some cases up to 5 years with treatment but is less than a month without it .
Although PCL is rare, the pathophysiology is classic and detection through a detailed physical exam and directed workup diagnosis can be promptly made, even when presenting as tamponade as did our patient. Patients presenting with signs and symptoms of new-onset acute decompensated heart failure can potentially have this diagnosis. Immediate detection is essential to avoid fatal complications, which include cardiac tamponade, ischemia, and fatal arrhythmia. To our knowledge, only 13 cases have described PCL presenting with tamponade physiology and are highlighted in Table 1. Parato et al. describe a case of a younger patient who presented with cardiac tamponade due to PCL. The patient was treated with management of cardiac tamponade, surgical excision of the mass, and chemotherapy. Complete remission was obtained after six months of chemotherapy . Although there was a large age difference, our patient also responded to chemotherapy following medical stabilization. Interestingly, Frikha et al. describe a case of an elderly immunocompromised patient presenting with cardiac tamponade along with paroxysmal third-degree atrioventricular (AV) block. They hypothesized that electrical compromise occurred via extension of the malignancy into the interatrial septum and the nodal tissue . Similarly, Gowda and Khan and Houchaymi et al. describe a case of an elderly immunocompetent male who was found to have pericardial tamponade along with third-degree AV block. However, pericardial fluid analysis did not reveal the diagnosis until a transvenous biopsy of the cardiac tumor revealed non-Hodgkin large B-cell lymphoma [7, 10]. Gowda and Khan and Chiba et al. report a similar case of a middle-aged male with cardiac tamponade and AV block who was found on right atrial tumor biopsy to have a diffuse large B-cell lymphoma. The patient underwent chemotherapy and required permanent pacemaker placement [7, 11]. On our extensive literature search, we identified one case report in which the cardiac tamponade was not caused by the lymphoma itself, but rather due to the malignant right atrial rupturing causing tamponade . Further cases are described in Table 1.
The etiology of PCL is still unknown, and molecular characterization has yet to be studied leaving a great deal of research to be done to gain a better understanding of this rare disease process. Nevertheless, although extremely rare, PCL can present with severe cardiac dysfunction due to tamponade physiology or impending tamponade physiology and heart failure. Clinical suspicion must remain high, and diagnostic studies of pericardial fluid can help prevent delay in management of an aggressive malignancy.
Conflicts of Interest
The authors declare that there is no conflict of interest.
 H. Ikeda, S. Nakamura, H. Nishimaki et al., "Primary lymphoma of the heart: case report and literature review," Pathology International, vol. 54, no. 3, pp. 187-195, 2004.
 G. L. Ceresoli, A. J. M. Ferreri, E. Bucci, C. Ripa, M. Ponzoni, and E. Villa, "Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management," Cancer, vol. 80, no. 8, pp. 1497-1506, 1997.
 C. S. Chim, A. C. L. Chan, Y. L. Kwong, and R. Liang, "Primary cardiac lymphoma," American Journal of Hematology, vol. 54, no. 1, pp. 79-83, 1997.
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 M. Duong, C. Dubois, M. Buisson et al., "Non-Hodgkin's lymphoma of the heart in patients infected with human immunodeficiency virus," Clinical Cardiology, vol. 20, no. 5, pp. 497-502, 1997.
 American Society of Clinical Oncology (ASCO), "Lymphoma non-Hodgkin: subtypes," 2016, https://www.cancer.net/ cancer-types/lymphoma-non-hodgkin/subtypes.
 R. M. Gowda and I. A. Khan, "Clinical perspectives of primary cardiac lymphoma," Angiology, vol. 54, no. 5, pp. 599-604, 2003.
 V. M. Parato, F. Muscente, and M. Scarano, "Primary cardiac lymphoma: a case report," Giornale Italiano di Cardiologia, vol. 18, no. 1,pp. 11-13, 2017.
 Z. Frikha, L. Abid, D. Abid et al., "Cardiac tamponade and paroxysmal third-degree atrioventricular block revealing a primary cardiac non-Hodgkin large B-cell lymphoma of the right ventricle: a case report," Journal of Medical Case Reports, vol. 5, no. 1, p. 433, 2011.
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 Y. Chiba, K. Oka, H. Saito, R. Nagayama, M. Murata, and N. Mori, "Primary cardiac B-cell lymphoma presented as heart tamponade and atrioventricular block: a case report," Acta Cytologica, vol. 54, no. 1, pp. 79-81, 2010.
 A. Menotti, F. Imperadore, G. Pelosi, and M. Disertori, "Heart rupture at the right atrial level as the first manifestation of malignant lymphoma," Cardiologia, vol. 41, no. 1, pp. 65-67, 1996.
Elisa Quiroz, (1) Adam Hafeez, (1) Ramy Mando, (1) Zhou Yu, (1) and Feroze Momin (2)
(1) Department of Internal Medicine, Beaumont Health, Royal Oak, MI, USA
(2) Department of Hematology and Oncology, Beaumont Health, Royal Oak, MI, USA
Correspondence should be addressed to Ramy Mando; email@example.com
Received 25 June 2018; Accepted 4 September 2018; Published 23 September 2018
Academic Editor: Jeanine M. Buchanich
Caption: Figure 1: Computed tomography of the chest demonstrating pericardial effusion.
Caption: Figure 2: Echocardiogram demonstrating a large circumferential effusion (outlined) concerning for impending tamponade.
Caption: Figure 3: Histopathology remarkable for monoclonal B-cells with significant large cell components.
Caption: Figure 4: Fluorescence in situ hybridization demonstrating t(8; 14). This translocation is between IGH on chromasome 8 and MYC on chromosome 14.
Table 1: Reports of primary cardiac lymphoma causing cardiac tamponade. Article (language, Description Highlights PMID) Parato VM, Muscente 35-year-old F, Scarano M. Primary immunocompromised cardiac lymphoma: a patient presented case report. G Ital with signs and Cardiol (Rome). 2017 symptoms of Jan; 18(1): 11-13 tamponade. (Italian, 28287210) Echocardiography revealed lateral AV mass with large pericardial effusion. Patient had surgical excision of mass followed by chemotherapy that induced complete remission at 6 months. Tzachanis D, Dewar R, 44-year-old female Primary Burkitt Luptakova K, Chang presented with lymphoma JD, et al. Primary abdominal bloating cardiac Burkitt who was found to have lymphoma presenting a large pericardial with abdominal pain effusion with (English, 25431699) tamponade physiology. Pericardial fluid studies led to diagnosis of cardiac Burkitt lymphoma. Frikha Z, Abid L, 64-year-old Primary non-Hodgkin Abid D, Mallek S, et immunocompromised large B-cell lymphoma al. Cardiac tamponade male presented with 3rd-degree AV block and paroxysmal third- cardiac tamponade as degree well as paroxysmal atrioventricular third-degree AV block revealing a block. He was found primary cardiac non- to have a large RV Hodgkin large B-cell mass. Following lymphoma of the right excision, histology ventricle: a case confirmed non- report. J Med Case Hodgkin large B-cell Rep. 2011 Sep 5; 5: lymphoma. 433 (English, 21892927) Houchaymi Z, Helou S, 78-year-old Primary non-Hodgkin Ballout J. immunocompetent male large B-cell lymphoma Pericardial tamponade presented with 3rd-degree AV block and third-degree pericardial tamponade atrioventricular and third-degree AV block revealing a block. Biopsy of the primary cardiac cardiac tumor showed lymphoma. Rev Med non-Hodgkin large B- Interne. 2010 Nov; 31 Cell lymphoma. (11): e4-6 (French, 20605278) Chiba Y, Oka K, Saito 49-year-old male with Diffuse large B-cell H, et al. Primary cardiac tamponade and lymphoma AV block cardiac B-cell AV block. Pericardial lymphoma presented as effusion and heart tamponade and transvenous biopsy atrioventricular confirmed diagnosis block: a case report. of diffuse large B- Acta Cytol. 2010 Jan- cell lymphoma. He Feb; 54 (1): 79-81 underwent (English, 20306995) chemotherapy and permanent pacemaker placement. Legault S, Couture C, 74-year-old male with Burkitt-like PCL Bourgault C, et al. dyspnea found to have Primary cardiac a large pericardial Burkitt-like lymphoma effusion with of the right atrium tamponade physiology. Found on pericardial fluid studies and biopsy to have Burkitt-like PCL. Ling LF, Chai P, Kee 55-year-old AC, et al. Primary immunocompetent man cardiac lymphoma presented with presenting with pyrexia initially and cardiac tamponade. Am was found later to Heart Hosp J. 2009 have cardiac Winter; 7 (2): E125- tamponade. He was 7 (English, 19279985) diagnosed with PCL. Gosev I, Siric F, 67-year-old Diffuse large B-cell Gasparovic H, et al. immunocompetent lymphoma of Surgical treatment of presented with centroblastic subtype a primary cardiac dyspnea. Found to Surgical diagnosis, lymphoma presenting have tamponade no pericardiocentesis with tamponade physiology on performed physiology (English, echocardiography. 16846425) Surgical biopsy confirmed diffuse large B-cell lymphoma of centroblastic subtype. Wilhite DB, Quigley 83-year-old Filipino Failure of RL. Occult cardiac male with dyspnea traditional lymphoma presenting found on pericardiostomy with cardiac echocardiogram to tamponade. Tex Heart have a large, Inst. J. 2003; 30 homogenous (1): 62-4 (English, pericardial effusion 12638674) with RA collapse. He was treated with subxiphoid pericardiostomy and had the pericardial drain removed the next day as he clinically improved. He returned two weeks later with same symptoms and again found to have reaccumulating pericardial fluid requiring urgent anterior pericardiectomy. Menotti A, Imperadore Malignant lymphoma Right atrial rupture F, Pelosi G. et al. presenting as cardiac causing the tamponade Heart rupture at the tamponade due to right atrial level as right atrial rupture. the first manifestation of malignant lymphoma. Cardiologia. 1996 Jan; 41(1):65-7 (Italian, 8697472) Roller MB, Manoharan Elderly male with A, Lvoff R. Primary cardiac tamponade cardiac lymphoma. found on pericardial Acta Haematol. 1991; fluid analysis to 85 (1): 47-48 have PCL. (English, 2011932) Nagamine K, Noda H. Two cases of males in Two cases cardiac their 70s presenting lymphoma presenting with dyspnea who were with pericardial found to have effusion and pericardial effusion tamponade. Jpn Circ. with tamponade J. 1990 Sep; 54 (9): physiology on 1158-64 (English, echocardiogram. 2266577) Pozniak AL, Thomas Patient who presented RD, Hobbs CB, et al. with ventricular Primary malignant arrhythmias and was lymphoma of the found to have heart. Antemorten pericardial effusion cytologic diagnosis. with impairment of LV Acta Cytol. 1986 Nov- contraction. Dec; 30 (6): 662-4. Postmortem (English, 3466501) examination showed malignant lymphoma confined to myocardium. Patel J, Melly L, Case series of six Clinical presentation Sheppard MN. Primary patients presenting of PCL (B-and T- cardiac lymphoma: B- with wide spectrum of cells) and T-cell cases at a symptoms. Most cases specialist UK centre involved 2 or more chambers of the heart. Presentations included conduction disturbances, effusion, valvular dysfunction, heart failure, stroke, and sudden death. Figure 5: Graphical representation of frequencies of subtypes of non-Hodgkin's lymphoma. Types of non -Hodgkin's lymphoma Primary Cardiac 10 Diffuse large B-cell 0.5 Follicular 20 Mantle cell 7 Small lymphocytic 5 Primary mediastinal 2.5 Lymphoplasmacytic 2 Marginal zone 10 Burkitt 0 T-cell lymphoma 2 Note: Table made from pie chart.
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|Title Annotation:||Case Report|
|Author:||Quiroz, Elisa; Hafeez, Adam; Mando, Ramy; Yu, Zhou; Momin, Feroze|
|Publication:||Case Reports in Oncological Medicine|
|Date:||Jan 1, 2018|
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