|Primary cardiac B-cell lymphoma with atrioventricular block and paroxysmal ventricular tachycardia.|
|Jump to Full Text|
|PMID: 22808929 Owner: NLM Status: MEDLINE|
|Primary cardiac lymphoma (PCL) is very rare, and is extremely challenging to diagnose due to nonspecific symptoms. When discovered, the right atrium and ventricle are most commonly affected, while diffuse cardiac involvement is uncommon. PCL is fatal unless promptly diagnosed and treated. Herein, we present the case of a 36-year-old immunocompetent male who presented with a 5-year history of non-specific chest symptoms and was diagnosed with primary diffuse cardiac large B-cell lymphoma involving the entire heart.|
|Ke-Wei Chen; Ju-Hsin Chang; Su-Peng Yeh; Chiung-Ray Lu|
Related Documents :
|15194979 - Diagnostic coronary arteriography in the interventional era: a combination of technical...
18764789 - Successful endovascular treatment of a hemodialysis graft pseudoaneurysm by covered ste...
10476589 - Usefulness of platelet glycoprotein iib/iiia inhibitors in coronary stenting for recons...
15883209 - Randomized comparison of a titanium-nitride-oxide-coated stent with a stainless steel s...
14597939 - Impact of lesion complexity on the capacity of a trial to detect differences in stent p...
17394249 - Stent thrombosis with drug-eluting stents: a re-examination of the evidence.
8025909 - Adenosine: a sensitive marker of myocardial ischaemia in man.
108989 - Variant angina pectoris: investigation of indexes of sympathetic nervous system function.
23131759 - Idiopathic ventricular fibrillation, early repolarization and other j wave-related vent...
|Type: Case Reports; Journal Article Date: 2012-07-18|
|Title: Journal of cardiothoracic surgery Volume: 7 ISSN: 1749-8090 ISO Abbreviation: J Cardiothorac Surg Publication Date: 2012|
|Created Date: 2012-07-31 Completed Date: 2013-01-30 Revised Date: 2013-07-12|
Medline Journal Info:
|Nlm Unique ID: 101265113 Medline TA: J Cardiothorac Surg Country: England|
|Languages: eng Pagination: 70 Citation Subset: IM|
|Division of Cardiology, Department of Internal Medicine, China Medical University Hospital, Taichung 40447, Taiwan.|
|APA/MLA Format Download EndNote Download BibTex|
Atrioventricular Block / pathology*
Heart Neoplasms / pathology*
Lymphoma, B-Cell / pathology*
Tachycardia, Ventricular / pathology*
Journal ID (nlm-ta): J Cardiothorac Surg
Journal ID (iso-abbrev): J Cardiothorac Surg
Publisher: BioMed Central
Copyright ©2012 Chen et al.; licensee BioMed Central Ltd.
Received Day: 14 Month: 12 Year: 2011
Accepted Day: 18 Month: 7 Year: 2012
collection publication date: Year: 2012
Electronic publication date: Day: 18 Month: 7 Year: 2012
Volume: 7First Page: 70 Last Page: 70
Publisher Id: 1749-8090-7-70
PubMed Id: 22808929
|Primary cardiac B-cell lymphoma with atrioventricular block and paroxysmal ventricular tachycardia|
|Ke-Wei Chen1||Email: firstname.lastname@example.org|
|Ju-Hsin Chang2||Email: email@example.com|
|Su-Peng Yeh3||Email: firstname.lastname@example.org|
|Chiung-Ray Lu1||Email: Drljr0821@gmail.com|
1Division of Cardiology, Department of Internal Medicine, China Medical University Hospital, 2 Yuh-Der Rd, Taichung, 40447, Taiwan
2Departments of Anesthesia, and Pain Service and Critical Care Medicine, China Medical University Hospital, Taichung, 40402, Taiwan
3China Medical University Hospital, Taichung, 40402, Taiwan
Primary cardiac lymphoma (PCL) is a very rare disorder that presents with nonspecific symptoms, making diagnosis challenging . The disorder accounts for approximately 1% of primary cardiac tumors and 0.5% of extranodal lymphomas, occurs three times more commonly in females than males with a median age at diagnosis of 64 years, and usually involves the right atrium and ventricle . We herein present the case of a 36-year-old immunocompetent male with a 5-year history of worsening chest symptoms who was diagnosed with a primary diffuse cardiac large B-cell lymphoma.
A 36-year-old immunocompetent male with history of gastroesophageal reflux presented with a 5-year history of dyspnea on exertion, chest tightness, shortness of breath, palpitations, and back pain. Physical exam revealed a heart murmur, ECG showed a Mobitz I AV block, RBBB, and right axis deviation, and chest radiograph demonstrated an enlarged heart. Laboratory studies were normal.
Transthoracic echocardiography revealed heterogeneous infiltration involving the aortic root and four chambers with marked biventricular wall thickening and moderate pericardial effusion (Figure 1A, B). The infiltrated myocardium stiffened the heart and significantly impaired systolic and diastolic functions. Computed tomography (CT) revealed a diffuse soft tissue density wrapping around the great vessels.
The patient was admitted and subsequently underwent endomyocardial biopsy prior to initiating treatment. Immunohistopathology showed infiltration of the myocardial fibers by atypical intermediate to large-sized lymphoid cell (Figure 2) that were positive for CD20, CD10, CD79, CD19, and LCA. Bone marrow biopsy revealed normocellular bone marrow, without evidence of lymphoma infiltrate. The final diagnosis was primary diffuse cardiac large B-cell lymphoma.
The patient subsequently received chemotherapy. The 1st cycle consisted of cyclophosphamide/vincristine/prednisolone (COP), omitting doxorubicin due to concern of heart toxicity given the heart block. Doxorubicin was added for the 2nd cycle (CHOP), and rituximab was added for the 3rd through 7th cycles (R-CHOP). CT showed diminished cardiac size after the first cycle. After the 2nd cycle, the patient developed ventricular tachycardia and received synchronized cardioversion with resultant 1st degree AV block. He then experienced paroxysmal ventricular tachycardia after the 4th cycle that responded to cardioversion.
The patient responded well to chemotherapy. ECG 6 months after treatment showed normal sinus rhythm. Follow-up echocardiogram demonstrated adequate global systolic and diastolic functions, with significant reduction of myocardial thickness and resolution of pericardial effusion (Figure 1C, D).
The incidence of primary cardiac tumors is approximately 0.02%, and PCLs account for approximately 1% of primary cardiac tumors and the majority is diffuse large B-cell lymphoma . A recent review of the literature by Miguel and Bestetti  for reports pertaining to the “clinical aspects, diagnosis, clinical course, or treatment” of PCL found 61 articles. Based on their review, the authors reported that PCL occurs more frequently in immunocompromised patients and those taking immunosuppressant drugs, cardiac transplant recipients, and predominantly male AIDS patients. In addition, PCL usually manifests after the fifth decades of life, and usually affects the right side of the heart. Though symptoms are non-specific and vary with the locations in the hearts affected, the most common clinical manifestations are pericardial effusion, heart failure, and AV-block . Diagnosis of PCL is often delayed due to non-specific symptoms, and the prognosis is poor with a reported median survival of 7 months after initiation of treatment [5,6], though improved survival has been seen with the addition of rituximab to chemotherapy regimens .
The initial symptoms of PCL are non-specific and can be suggestive of many disorders including heart failure, infection, arrhythmias, and emboli . Though PCL is typically seen in older patients and is associated with an immunocompromised state [1,2], our case illustrates that the condition can occur in young, otherwise healthy individuals. Interestingly, our patient presented with diffuse cardiac infiltration involving the entire heart, rather than the more commonly reported presentation involving single site such as the right atrium/ventricle [1,2].
Imaging modalities including echocardiography, CT, and magnetic resonance imaging (MRI) are useful for illustrating the cardiac involvement in PCL; however, histopathological examination of biopsied or surgical specimen is needed for diagnosis . While in the past PCL was almost universally fatal, advances in imaging and anthracycline-based chemotherapy have markedly improved survival, though the median survival remains low .
Our patient presented with Mobitz I AV block and RBBB and developed several arrhythmias during treatment. The myocardium infiltrated by lymphoma cells becomes heterogeneous substrate, which is prone to develop cardiac arrhythmias or conduction disorders . Special attention should be given to high-grade AV blocks or fatal arrhythmias during treatment of PCL . Unfortunately, indications for placement of pacemaker or implantable cardioverter defibrillator in patients with cardiac lymphomas are not straightforward . Given the high mortality of PCL, and resolution of conduction disorders and arrhythmias with treatment, device placement is not likely warranted in most cases. Though rare, cardiac rupture can occur with diffuse cardiac involvement and should be monitored .
In summary, PCL can occur in young healthy individuals and should be considered in the differential diagnosis when treatments for more common conditions fail. Heart block and arrhythmias in PCL with diffuse cardiac involvement can be severe, and warrant additional vigilance during management.
Written informed consent was obtained from the patient for publication of this report and any accompanying images.
The authors declare that they have no competing interests
KWC drafted the manuscript, analysed and interpreted the echocardiography. JHC participated in the sequence alignment and drafted the manuscript. SPY participated in the sequence alignment and drafted the manuscript, analysed and interpreted the pathologic image. CRL participated in the sequence alignment and drafted the manuscript, analysed and interpreted the echocardiography. All authors read and approved the final manuscript.
|Gowda RM,Khan IA,Clinical perspectives of primary cardiac lymphomaAngiologyYear: 20035459960410.1177/00033197030540051014565636|
|Sheppard MN,Angelini A,Raad M,Savelieva I,Camm AJF, Luscher T, Serruys PWTumours of the heartThe ESC Textbook of Cardiovascular MedicineYear: 2006Blackwell, London535552|
|Reynen K,Frequency of primary tumors of the heartAm J CardiolYear: 19967710710.1016/S0002-9149(97)89149-78540447|
|Miguel CE,Bestetti RB,Primary cardiac lymphomaInt J CardiolYear: 201114935836310.1016/j.ijcard.2010.02.01620227122|
|Miyashita T,Miyzawa I,Kiyosawa K,A case of primary cardiac B cell lymphoma associated with ventricular tachycardia, successfully treated with systemic chemotherapy and radiotherapyJpn Circ JYear: 20006413513810.1253/jcj.64.13510716528|
|Mejhert M,Muller-Suur,Primary lymphoma of the heartScand Cardiovasc JYear: 20003460660810.1080/14017430075006457611214017|
|Tanaka Y,Yamabe H,Yamasaki H,Tsuda H,Nagayoshi Y,Kawano H,Kimura Y,Hokamura Y,Ogawa H,A case of reversible ventricular tachycardia and complete atrioventricular block associated with primary cardiac B-cell lymphomaPacing Clin ElectrophysiolYear: 20093281681910.1111/j.1540-8159.2009.02372.x19545348|
|Dellas C,Chapuy B,Schweyer S,Hasenfuss G,Hünlich M,A rare cause of sudden cardiac arrest: primary cardiac lymphomaClin Res CardiolYear: 20099850951110.1007/s00392-009-0034-019499163|
|Armstrong EJ,Bhave P,Wong D,Ursell PC,Kaplan L,Yeghiazarians Y,Ai WZ,Left ventricular rupture due to HIV-associated T-cell lymphomaTex Heart Inst JYear: 20103745746020844622|
Keywords: Primary cardiac lymphoma, AV block, PVT.
Previous Document: Nitrogen dioxide levels estimated from land use regression models several years apart and associatio...
Next Document: A Genome-wide Regulator-DNA Interaction Network in the human pathogen Mycobacterium tuberculosis H37...