© 1991 S. Karger AG, Basel 0001 -5792/91/0851 -0047 S 2.75/0
Acta Haematol 1991;85:47-48
Primary Cardiac Lymphoma M.B. Roller', A. Mcmoharanb, R. L voffa Departments of a Cardiology and b Clinical Haematology, St. George Hospital, Kogarah, Australia
Key Words. Cardiac lymphoma • Primary tumour Abstract. An elderly man presented with pericardial tamponade: a diagnosis of primary cardiac lymphoma was made by a cytological examination of pericardial fluid. An excellent response to therapy has been ob served.
Primary localised cardiac lymphomas (those in volving only the heart and pericardium) are extremely rare tumours, with only 20 reported cases in the litera ture so far [1, 2]. We present a case of primary cardiac lymphoma and a review of the literature. Case Report A previously well 82-year-old man presented with a 2-week his tory of epigastric discomfort, nausea and dyspnoea. On examina tion he was in sinus rhythm with occasional extrasystoles. Blood pressure was normal (140/90 mm Hg). Cardiovascular and respiratory examination was otherwise normal, and no lymphadenopathy or hepatosplenomegaly was present. The initial chest X-ray showed cardiomegaly and a small left pleural effusion; an electrocardiogram revealed sinus rhythm with atrial extrasystoles. The full blood count was normal: haemo globin 14.2 g/dl, white cell count 5.4 x 109/1 and platelet count 219xl09/l. Serum urea and creatinine were raised: urea 12.0 mmol/1 and creatinine 0.15 mmol/1. Urine microscopy was nor mal, and a renal ultrasound was also normal. Urinary urea, creati nine and electrolytes suggested a prerenal cause of renal dysfunc tion. Oral and then intravenous fluids were administered. The pa tient developed sacral and ankle oedema, the jugular venous pres sure became elevated and atrial fibrillation supervened. Renal function worsened, and the pattern of renal dysfunction changed to one of acute tubular necrosis. A two-dimensional echocardiogram was performed, and this showed a large pericardial effusion with evidence of right heart compression, consistent with cardiac tamponade. Pericardiocente sis was performed, yielding 500 ml of heavily blood-stained fluid
resulting in symptomatic and clinical improvement, this procedure was repeated on two subsequent occasions, and the patient’s prog ress was assessed by serial echocardiograms. During this period the patient’s renal function returned to normal, and his general condi tion improved. The cytology of the pericardial aspirate was consis tent with lymphomatous infiltration (fig. 1), and the immunocytochemistry of the cells revealed a monoclonal B cell proliferation (IgMK). A microbiological examination of the fluid was negative for bacterial and mycobacterial infections. Bone marrow aspiration and trephine biopsy were normal, and thoraco-abdominal computed tomography scan examination showed no evidence of lymphoma outside the pericardium; a gal lium scan showed only a pericardial rim of tracer uptake. The patient was treated with 60 mg prednisolone daily and a course of local radiotherapy (total dose 20 Gy). Prednisolone was tapered off over the next 6 months. The patient has been well and disease free for 15 months.
Discussion A review of the 21 reported cases of primary car diac lymphoma shows that there are no symptoms or signs that particularly suggest the diagnosis, and clini cal symptoms may be entirely absent (6 cases) [1, 3]; the patient may present with features of congestive heart failure (9 cases) [1,4, 5) or pericardial effusion (8 cases) [1, our case]; 1 patient each presented with sudden death [1] and chest pain with third-degree atrioventricular block [6]. Sixteen of the 21 patients were only diagnosed post mortem. Of the 5 diagnosed ante mortem, 2 were diagnosed by myocardial biopsy alone [1, 5], 2 were diagnosed by pericardiocentesis
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Introduction
Roller/M anoharan/Lvoff
48
A further point of interest is the recently reported association between the immunocompromised state and the development of primary cardiac lymphoma; 3 patients were noted to have HIV infection [3, 4] while the case reported by Rodenburg et al. [2] occurred in a renal transplant recipient. Acknowledgement We thank Mrs. Margaret Jenkins for typing the manuscirpt.
References
alone [1, our case] and 1 was diagnosed by both [6], Three of the 5 patients in whom an ante-mortem diag nosis was made died before treatment could be com menced or soon after due to lethal local complica tions of the disease [1,5,7]. The varying histological classifications and the in complete nature of some of the earlier reports pre clude a meaningful interpretation of the pathology and correlation with the clinical course. The present case is 1 of only 2 in which treatment has been insti tuted with a successful outcome - the case reported by Castelli et al. [6] received combination chemotherapy CHOP (cyclophosphamide, Adriamycin, vincristine, prednisolone). Gallium scanning is now widely used as a diagnos tic and staging investigation in patients with nonHodgkin’s lymphoma [8]. Our experience and that of others [4,7] confirm the useful role of gallium-67 scan examination in primary cardiac lymphoma, espe cially to rule out disseminated disease in patients with a positive pericardial uptake.
1 Curtslnger CR, Wilson MJ, Yoneda K: Primary cardiac lym phoma. Cancer 1989;64:521-525. 2 Rodenburg CJ, Kluin P, Maes A, Paul LC: Malignant lym phoma confined to the heart, 13 years after a cadaver kidney transplant. N Engl J Med 1985:313:122. 3 Guarner J, Brynes RK, Chan WC, Birdsong G, Hertzler G: Pri mary non-Hodgkin’s lymphoma of the heart in two patients with the acquired immunodeficiency syndrome. Arch Pathol Lab Med 1987;111:254-256. 4 Constantino A, West TE, Gupta M, Loghmanee F: Primary cardiac lymphoma in a patient with acquired immunodefi ciency syndrome. Cancer 1987;60:2801-2805. 5 Proctor MS, Tracy GP, Von Koch L: Primary cardiac B-cell lymphoma. Am Heart J. 1989; 118:179-181. 6 Castelli MJ, Mihalov ML, Posniak HV, Gattuso P: Primary cardiac lymphoma initially diagnosed by routine cytology. Acta Cytol 1989:33:355-358. 7 Chou ST, Arkles LB, Gill GD, Pinkus N, Parkin A, Hicks JD: Primary lymphoma of the heart. A case report. Cancer 1983; 52:744-747. 8 Anderson KC, Leonard RCF, Canellos GP: High dose gallium imaging in lymphoma. Am J Med 1983;75:327-331.
Received: June 26,1990 Accepted: July 8,1990 Arumugam Manoharan St. George Hospital Kogarah, NSW 2217 (Australia)
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 7/8/2018 10:23:28 PM
Fig. 1. Pericardial fluid cytology showing malignant lymphoma cells, some with vacuoles, x 40.
Acta Haematol 1991;85:47-48
Primary Cardiac Lymphoma M.B. Roller', A. Mcmoharanb, R. L voffa Departments of a Cardiology and b Clinical Haematology, St. George Hospital, Kogarah, Australia
Key Words. Cardiac lymphoma • Primary tumour Abstract. An elderly man presented with pericardial tamponade: a diagnosis of primary cardiac lymphoma was made by a cytological examination of pericardial fluid. An excellent response to therapy has been ob served.
Primary localised cardiac lymphomas (those in volving only the heart and pericardium) are extremely rare tumours, with only 20 reported cases in the litera ture so far [1, 2]. We present a case of primary cardiac lymphoma and a review of the literature. Case Report A previously well 82-year-old man presented with a 2-week his tory of epigastric discomfort, nausea and dyspnoea. On examina tion he was in sinus rhythm with occasional extrasystoles. Blood pressure was normal (140/90 mm Hg). Cardiovascular and respiratory examination was otherwise normal, and no lymphadenopathy or hepatosplenomegaly was present. The initial chest X-ray showed cardiomegaly and a small left pleural effusion; an electrocardiogram revealed sinus rhythm with atrial extrasystoles. The full blood count was normal: haemo globin 14.2 g/dl, white cell count 5.4 x 109/1 and platelet count 219xl09/l. Serum urea and creatinine were raised: urea 12.0 mmol/1 and creatinine 0.15 mmol/1. Urine microscopy was nor mal, and a renal ultrasound was also normal. Urinary urea, creati nine and electrolytes suggested a prerenal cause of renal dysfunc tion. Oral and then intravenous fluids were administered. The pa tient developed sacral and ankle oedema, the jugular venous pres sure became elevated and atrial fibrillation supervened. Renal function worsened, and the pattern of renal dysfunction changed to one of acute tubular necrosis. A two-dimensional echocardiogram was performed, and this showed a large pericardial effusion with evidence of right heart compression, consistent with cardiac tamponade. Pericardiocente sis was performed, yielding 500 ml of heavily blood-stained fluid
resulting in symptomatic and clinical improvement, this procedure was repeated on two subsequent occasions, and the patient’s prog ress was assessed by serial echocardiograms. During this period the patient’s renal function returned to normal, and his general condi tion improved. The cytology of the pericardial aspirate was consis tent with lymphomatous infiltration (fig. 1), and the immunocytochemistry of the cells revealed a monoclonal B cell proliferation (IgMK). A microbiological examination of the fluid was negative for bacterial and mycobacterial infections. Bone marrow aspiration and trephine biopsy were normal, and thoraco-abdominal computed tomography scan examination showed no evidence of lymphoma outside the pericardium; a gal lium scan showed only a pericardial rim of tracer uptake. The patient was treated with 60 mg prednisolone daily and a course of local radiotherapy (total dose 20 Gy). Prednisolone was tapered off over the next 6 months. The patient has been well and disease free for 15 months.
Discussion A review of the 21 reported cases of primary car diac lymphoma shows that there are no symptoms or signs that particularly suggest the diagnosis, and clini cal symptoms may be entirely absent (6 cases) [1, 3]; the patient may present with features of congestive heart failure (9 cases) [1,4, 5) or pericardial effusion (8 cases) [1, our case]; 1 patient each presented with sudden death [1] and chest pain with third-degree atrioventricular block [6]. Sixteen of the 21 patients were only diagnosed post mortem. Of the 5 diagnosed ante mortem, 2 were diagnosed by myocardial biopsy alone [1, 5], 2 were diagnosed by pericardiocentesis
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 7/8/2018 10:23:28 PM
Introduction
Roller/M anoharan/Lvoff
48
A further point of interest is the recently reported association between the immunocompromised state and the development of primary cardiac lymphoma; 3 patients were noted to have HIV infection [3, 4] while the case reported by Rodenburg et al. [2] occurred in a renal transplant recipient. Acknowledgement We thank Mrs. Margaret Jenkins for typing the manuscirpt.
References
alone [1, our case] and 1 was diagnosed by both [6], Three of the 5 patients in whom an ante-mortem diag nosis was made died before treatment could be com menced or soon after due to lethal local complica tions of the disease [1,5,7]. The varying histological classifications and the in complete nature of some of the earlier reports pre clude a meaningful interpretation of the pathology and correlation with the clinical course. The present case is 1 of only 2 in which treatment has been insti tuted with a successful outcome - the case reported by Castelli et al. [6] received combination chemotherapy CHOP (cyclophosphamide, Adriamycin, vincristine, prednisolone). Gallium scanning is now widely used as a diagnos tic and staging investigation in patients with nonHodgkin’s lymphoma [8]. Our experience and that of others [4,7] confirm the useful role of gallium-67 scan examination in primary cardiac lymphoma, espe cially to rule out disseminated disease in patients with a positive pericardial uptake.
1 Curtslnger CR, Wilson MJ, Yoneda K: Primary cardiac lym phoma. Cancer 1989;64:521-525. 2 Rodenburg CJ, Kluin P, Maes A, Paul LC: Malignant lym phoma confined to the heart, 13 years after a cadaver kidney transplant. N Engl J Med 1985:313:122. 3 Guarner J, Brynes RK, Chan WC, Birdsong G, Hertzler G: Pri mary non-Hodgkin’s lymphoma of the heart in two patients with the acquired immunodeficiency syndrome. Arch Pathol Lab Med 1987;111:254-256. 4 Constantino A, West TE, Gupta M, Loghmanee F: Primary cardiac lymphoma in a patient with acquired immunodefi ciency syndrome. Cancer 1987;60:2801-2805. 5 Proctor MS, Tracy GP, Von Koch L: Primary cardiac B-cell lymphoma. Am Heart J. 1989; 118:179-181. 6 Castelli MJ, Mihalov ML, Posniak HV, Gattuso P: Primary cardiac lymphoma initially diagnosed by routine cytology. Acta Cytol 1989:33:355-358. 7 Chou ST, Arkles LB, Gill GD, Pinkus N, Parkin A, Hicks JD: Primary lymphoma of the heart. A case report. Cancer 1983; 52:744-747. 8 Anderson KC, Leonard RCF, Canellos GP: High dose gallium imaging in lymphoma. Am J Med 1983;75:327-331.
Received: June 26,1990 Accepted: July 8,1990 Arumugam Manoharan St. George Hospital Kogarah, NSW 2217 (Australia)
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 7/8/2018 10:23:28 PM
Fig. 1. Pericardial fluid cytology showing malignant lymphoma cells, some with vacuoles, x 40.
