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Immunoblastic Lymphoma Presenting with Syncope Due to Sinoatrial Node Disease

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Immunoblastic Lymphoma Presenting with Syncope Due to Sinoatrial Node Disease Postgrad Med J: first published as 10.1136/pgmj.66.775.395 on 1 May 1990. Downloaded from Postgrad Med J (1990) 66, 395 397 The Fellowship of Postgraduate Medicine, 1990 Immunoblastic lymphoma presenting with syncope due to sinoatrial node disease G. Dolan, A.P. Jones and J.T. Reilly. Department ofHaematology, Northern General Hospital, Herries Road, Sheffield S5 7AJ, UK. Summary: A 68 year old man presented with syncope associated with episodes of sinus arrest. He responded to insertion of a demand pacemaker. During investigation he was noted to have generalized lymphadenopathy and biopsy revealed that he had an immunoblastic lymphoma. Before further assessment could be made, he developed rapid atrial fibrillation, resistant to several anti-arrhythmic agents and died several days after admission. At autopsy a nodular plaque of lymphoma was found to overly the sinoatrial node which was markedly fibrosed with evidence of lymphomatous infiltration of the surrounding microvasculature. Introduction Cardiac involvement by lymphoma is well recog- arrest. He thus had a permanent demand nized but is usually asymptomatic. The unusual pacemaker implanted and no further episodes presentation described in this case illustrates the occurred. importance ofconsidering the possibility of metas- A diagnosis of immunoblastic lymphoma was copyright. tases when new symptoms of cardiac disease arise made from lymph node biopsy but before further in patients with lymphoma, as therapeutic assessment could be arranged, the patient develop- intervention may be possible. ed paroxysmal rapid atrial fibrillation associated with hypotension. These episodes of fibrillation became prolonged and failed to respond to intra- Case report venous digoxin, verapamil or amiodarone infusion. The patient developed progressive cardiac and A 68 year old man presented with a 2-month renal failure and despite intensive support http://pmj.bmj.com/ history of blackouts lasting 10-15 seconds. On measures he deteriorated rapidly and died several examination he was in sinus rhythm with a blood days after admission. pressure of 130/70 mmHg. He was noted, however, At autopsy, there was marked intra-abdominal to have generalized lymphadenopathy, hepato- lymphadenopathy and also involvement of the splenomegaly and a widespread maculopapular kidneys and heart by lymphoma. Closer examina- rash. Resting electrocardiograph, electrolytes and tion of the heart revealed that there was a liver function tests were normal but he had a 1.5 cm x 1 cm lymphomatous plaque on the normochromic, normocytic anaemia of 82 g/l. epicardial surface of the heart and a I cm x I cm on September 29, 2021 by guest. Protected During admission for further investigation, in- plaque on the superior vena cava. There was a cluding lymph node biopsy, he suffered an acute, 1 cm x 1 cm nodular deposit of lymphoma overly- transient episode of unconsciousness associated ing the sinoatrial (SA) node (Figure 1). Histological with extreme pallor, pupillary dilatation and disap- sections through this area showed that, although pearance of peripheral pulses. The patient made a there was no evidence of direct invasion of the spontaneous, complete recovery within 10 seconds. neural tissue by lymphoma, there was a diffuse A provisional diagnosis of Stokes-Adams attack infiltrate of malignant cells in the tissue and small was made and urgent 48 hour electrocardiogram blood vessels surrounding the SA node (Figure 2), (ECG) monitoring was arranged. Two similar which was markedly fibrosed. The coronary episodes occurred during the next 48 hours, both arteries showed only a modest degree of atheroma were shown to coincide with short episodes ofsinus and there was no other significant evidence of ischaemic heart disease. These appearances were felt to be compatible with local ischaemic damage to the SA node due to Correspondence: G. Dolan M.B., Ch.B., M.R.C.P. diffuse involvement of the surrounding micro- Accepted: 3 January 1990 vasculature. Postgrad Med J: first published as 10.1136/pgmj.66.775.395 on 1 May 1990. Downloaded from 396 CLINICAL REPORTS found that 48 had evidence of cardiac involve- ment.4 Analysis of the various subtypes of lym- phoma revealed that 16% of Hodgkin's disease, 25% of non-Hodgkin's lymphoma and 33% of cases of mycosis fungoides had metastatic disease of the heart. The majority of cases of secondary lymphoma of the heart are clinically silent. The most common clinical manifestation appears to be pericardial effusion with tamponade.' Although a variety of rhythm disturbances including atrial tachycardias"5 and heart block6'7 have been des- cribed, these may be more commonly due to associated factors such as electrolyte disturbance, hypoxia or cardiotoxic chemotherapy.4 Cardiac dysfunction due to secondary lym- phoma is very rarely the presenting feature of the disease.1"2 Cole et al.7 described a boy with Burkitt's lymphoma who presented with complete heart block associated with atrioventricular node involvement and Goggio et al.8 described a case of Hodgkin's disease presenting with Stokes-Adams syndrome. .... We present the unusual presentation of Lzit I~~~~~~~~~~~~~~~~....... immunoblastic lymphoma with syncope associated with sinus arrest due to ischaemic damage and Cm fibrosis of the sinoatrial node. Involvement of the Figure 1 Showing nodular deposit oflymphoma overly- sinoatrial node associated with rhythm disturbance copyright. ing the SA node. has been previously noted in non-Hodgkin's lym- phoma,5 though not, to our knowledge, as a Discussion presenting feature. It is important to consider the possibility of Metastatic tumours of the heart are more common secondary cardiac involvement in those patients than primary tumours.'"2 Bronchogenic and breast with malignant disease who have evidence of carcinomas, malignant melanomas and lym- cardiac dysfunction. Treatment with anti- phomas are those which more commonly give rise arrhythmic therapy may be unsuccessful in such http://pmj.bmj.com/ to cardiac involvement.3 cases, although response to cytotoxic therapy may, A study of 196 patients dying with lymphoma at least in theory, be possible. on September 29, 2021 by guest. Protected Figure 2 High power view showing a diffuse infiltrate ofimmunoblastic lymphoma in the myocardium around the SA node. A small blood vessel is also infiltrated. (H & E x 625). Postgrad Med J: first published as 10.1136/pgmj.66.775.395 on 1 May 1990. Downloaded from CLINICAL REPORTS 397 References 1. Rosenthal, D.S. & Braunwald, E. Haematologic-oncologic 5. Allen, D.C., Allerdice, J.M., Morton, P., Mollan, R.A.B. & disorders and heart disease. In: Braunwald, E. (ed) Heart Morris, T.C.M. Pathology ofthe heart and conduction system Disease. A Textbook of Cardiovascular Medicine. W.B. in lymphoma and leukaemia. J Clin Pathol 1987, 40: 746-750. Saunders, Philadelphia, 1987, pp. 1734-1757. 6. Kellaway, G. & Gardner, D.L. Metastatic reticulum cell 2. Hall, R.J. & Cooley, D.A. Neoplastic heart disease. In: Hurst, sarcoma ofthe heart causing complete heart block. Scott Med J.W. (ed) The Heart. McGraw-Hill, New York, 1982, J 1959, 4: 575-580. pp. 1403-1424. 7. Cole, T.O., Attah, E.B. & Onyemelukwe, G.C. Burkitt's 3. Kapoor, A.S. Clinical manifestations ofneoplasia ofthe heart. lymphoma presenting with heart block. Br Med J 1975, 37: In: Kapoor, A.S. (ed) Cancer and the Heart. Springer-Verlag, 94-97. New York, 1986, pp. 21-25. 8. Goggio, A.F., Harkness, J.T. & Palmer, W.S. Stokes-Adams 4. Roberts, W.C., Glancy, D.L. & De Vita, V.T. Heart in syndrome in Hodgkin's granuloma. JAMA 1961, 176: malignant lymphoma (Hodgkin's diseagse, lymphomosarcoma, 687-689. reticulum cell sarcoma and mycosis fungoides): a study of 196 autopsy cases. Am J Cardiol 1968, 22: 85-107. copyright. http://pmj.bmj.com/ on September 29, 2021 by guest. Protected.
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