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Myocardial necrosis in light chain deposition
Volume 110 Number 6
Rrief
anterior descending artery at its most distal portion. Biopsy proved tumor embolus at this point. The fact that the patient suffered an extensive anterior myocardial infarction indicates migration of the tumor (following the presenting event) to its more distal position at operation. Previous reportslo.ll have shown similar findings in the coronary and cerebral circulation, with the area of embolization at time of diagnosis distal to where the occlusion must have been to account for the presenting symptoms. In summary, a patient with an acute anterior myocardial infarction, resulting from tumor embolus from a left atria1 myxoma to the left anterior descending coronary artery, is presented. The clinical clues prompting echocardiography were a loud first heart sound and a short presystolic murmur. This is the first report of acute myocardial infarction as the presenting symptom of atrial myxoma in which the diagnosis was established prior to catheterization. The importance of prompt surgery for removal of the tumor mass is emphasized, as there is a high incidence of recurrent emboli which may prove to be life-threatening. REFERENCES
1. McAllister 2. 3.
4.
9. 10.
11.
HA: Primary tumors and cysts of the heart and pericardium. Curr Probl Cardiol 4:2, 1979. Wenger NK, Bauer S: Coronary embolism. Am J Med 26549, 1958. Chamberlin SW, Carter JR, Richardson RL: Intraoperative coronary artery embolization from left atria1 myxoma. Anesthesiology 47:301, 1977. Meller J, Teicholz LE, Richard AD, Matta R, Litwak R, Herman MV: Left ventricular myxoma. Echocardiographic diagnosis and review of the literature. Am J Med 63:816, 1977. Silverman J, Olwin J, Graettinger J: Cardiac myxomas with systemic embolization. Circulation 26:99, 1962. Burton C, Johnston J: Multiple cerebral aneurysms and cardiac myxoma. N Engl J Med 282:35, 1970. Sybers HD, Boake WC: Coronary and retinal embolism from left atria1 myxoma. Arch Path01 91:179, 1971. Tanabe J, Williams RL, Dietrich EB: Left atria1 myxoma: Association with acute coronary embolization in an 11 yearold boy. Pediatrics 63:778, 1979. Price DL, Harris JL, New PFJ, Canty RC: Cardiac myxoma. Arch Neurol 23:558, 1971. Differding JT, Gardner RE, Roe BB: Intracardiac myxomas with report of two unusual cases and successful removal. Circulation 23:929, 1961. Balk AHM, Wagenaar SS, Bruschke AVG: Bilateral cardia myxomas and peripheral myxomas in a patient with recent myocardial infarction. Am J Cardiol 44:767, 1979.
Myocardial deposition
Communications
1295
Systemic light chain deposition is a rare finding in patients with plasma cell dyscrasias. Light chains can produce renal tubular damage and occasional glomerular abnormalities.‘*2 but except for certain forms of amyloidosis, widespread deposition of light chains is uncommon.3, 4 This case report describes systemic light chain deposition and associated myocardial coagulative necrosis without morphologic evidence of amyloidosis. Myocardial cell death has not previously been associated with plasma cell dyscrasias and systemic light chain deposition. A 35-year-old woman was referred to Hahnemann University Hospital with a 4-month history of weakness and fatigue. A workup begun at an outside hospital had revealed renal failure, acidosis, and an elevated serum calcium level. The patient had been started on hemodialysis and an elevated serum parathyroid hormone suggested primary hyperparathyroidism. Three and one-half parathyroids were surgically excised and were described as being hyperplastic on pathologic examination. Nonetheless, serum calcium levels remained elevated after parathyroidectomy. Physical examination revealed that the patient was disoriented as to time and place. The blood pressure was 100/60 mm Hg, the pulse was 98 bpm, the temperature was 100.6’ F, and the respirations were 22lmin. A right central facial palsy was present, and the remainder of the physical examination was unremarkable.
necrosis in light chain
Eric Staros, M.D., and Sheila Moriber Katz, M.D. Philadelphia,
Pa.
From the Department of Pathology University School of Medicine.
Reprint mann
requests: University,
and Laboratory
Medicine,
Hahnemann
Sheila Moriber Katz, M.D., Dept. of Pathology, HahneBroad & Vine Streets, Philadelphia, PA 19102.
Fig. 1. Electron micrograph of myocardium. Dense deposits are seen along the myocardial cell basement membrane and in the interstitial space (arrows). (Lead citrate, uranyl acetate; original magnification ~23,000.)
1296
December, 1985 American Heart Journal
Brief Communications
Fig. 2. The upper frame is a hematoxylin and eosin preparation which shows coagulative necrosis and cellular loss. The remaining interstitial network is thickened (original magnification x350). The lower frame shows kappa light chain fluorescent staining of the interstitium (arrow).
Additional studies included a normal chest x-ray examination, a calcium level of 13.5 mg/dl (normal greater than 9.2 and less than 11.0 mg/dl), and elevated liver enzymes as follows: serum glutamic oxaloacetic transaminase @GOT) 56 U/L (normal <34), alkaline phosphatase 372 (U/L (normal <74), and lactate dehydrogenase (LDH) 299 U/L (normal <196). The serum creatinine was 3.8 mg/dl (normal
cardiopathy. Despite the use of pressor agents, the patient became increasingly hypotensive and died. At autopsy, the heart weighed 520 gm and had a rubbery consistency. The coronary arteries were patent. Over visceral organs, most notably the lungs, spleen, kidneys and liver, were heavy and exhibited a similar firm consistency. On microscopic examination, the heart showed extensive regions of transmural myocytolysis, moderately thickened intramural coronary arteries, and the presence of homogeneous, eosinophilic interstitial tissue. Electron microscopy showed intramembranous dense deposits along myocardial and endothelial basement membranes. Immunofluorescent staining of the heart for kappa light chains was positive, while control sera for lambda light chain, fibrin, and complement compounds, were negative. Examination of the liver, pericardium, kidney, and breast showed intramembranous dense deposits along endothelial basement membranes by electron microscopic examination. The kidneys contained occasional glomeruli showing focal nodular sclerosis and multinucleated tubular epithelial cells, findings which are specific for light chain disease. Congo red stains for the detection of amyloid were negative and no fibrillar deposits were seen by electron microscopy. The bone marrow contained a moderately differentiated plasma cell infiltrate which stained for kappa chains by immunoperoxidase techniques. Dense deposits which are fluorescent when stained with fluorescein conjugated anti-light chain antisera are diagnostic for light chain deposition.5 While renal tubular damage and nodular sclerosis of glomeruli have been well described in light chain disease, systemic deposition is rare. Though mild congestive heart failure and nonspecific ECG changes have been described in association with systemic light chain deposition,5 the histopathologic findings of myocardial light chain disease have not been previously described. This case demonstrates an association between light chain disease and myocardial necrosis6 Because the patent coronary arteries exclude atherosclerosis as a cause of myocardial ischemia, necrosis appears to be a consequence of a cellular injury which was presumably caused by light chain deposition in intramural coronary arteries and the myocardial interstitium. REFERENCES
Bryan CW, Healy JK: Acute renal failure in multiple myeloma. Am J Med 44:128, 1968. 2. Levi DF, Williams RC Jr, Lindstrom FD: Immunofluorescent 1.
3.
4.
5.
6.
studies of the myeloma kidney with special references to light chain disease. Am J Med 44:922, 1968. Glenner GG, Ein D, Eanes ED, Bladen HA, Teny W, Page DL: Creation of “amyloid” fibrils from Bence Jones proteins in vitro. Science 174:712, 1971. Shirahama T, Benson MD, Cohen AS, Tamaka A: Fibrillar assemblage of variable segments of immunoglobulin light chains. J Immunol 110:21, 1973. Randall RE, Williamson WC, Mullinax F, Tung MY, Still WJS: Manifestations of systemic light chain deposition. Am J Med 60:29X 1976. Baroldi G: Different types of myocardial necrosis in coronary heart disease: Pathophysiologic review of functional significance. AM HEART J 89:742, 1975.
Rrief
anterior descending artery at its most distal portion. Biopsy proved tumor embolus at this point. The fact that the patient suffered an extensive anterior myocardial infarction indicates migration of the tumor (following the presenting event) to its more distal position at operation. Previous reportslo.ll have shown similar findings in the coronary and cerebral circulation, with the area of embolization at time of diagnosis distal to where the occlusion must have been to account for the presenting symptoms. In summary, a patient with an acute anterior myocardial infarction, resulting from tumor embolus from a left atria1 myxoma to the left anterior descending coronary artery, is presented. The clinical clues prompting echocardiography were a loud first heart sound and a short presystolic murmur. This is the first report of acute myocardial infarction as the presenting symptom of atrial myxoma in which the diagnosis was established prior to catheterization. The importance of prompt surgery for removal of the tumor mass is emphasized, as there is a high incidence of recurrent emboli which may prove to be life-threatening. REFERENCES
1. McAllister 2. 3.
4.
9. 10.
11.
HA: Primary tumors and cysts of the heart and pericardium. Curr Probl Cardiol 4:2, 1979. Wenger NK, Bauer S: Coronary embolism. Am J Med 26549, 1958. Chamberlin SW, Carter JR, Richardson RL: Intraoperative coronary artery embolization from left atria1 myxoma. Anesthesiology 47:301, 1977. Meller J, Teicholz LE, Richard AD, Matta R, Litwak R, Herman MV: Left ventricular myxoma. Echocardiographic diagnosis and review of the literature. Am J Med 63:816, 1977. Silverman J, Olwin J, Graettinger J: Cardiac myxomas with systemic embolization. Circulation 26:99, 1962. Burton C, Johnston J: Multiple cerebral aneurysms and cardiac myxoma. N Engl J Med 282:35, 1970. Sybers HD, Boake WC: Coronary and retinal embolism from left atria1 myxoma. Arch Path01 91:179, 1971. Tanabe J, Williams RL, Dietrich EB: Left atria1 myxoma: Association with acute coronary embolization in an 11 yearold boy. Pediatrics 63:778, 1979. Price DL, Harris JL, New PFJ, Canty RC: Cardiac myxoma. Arch Neurol 23:558, 1971. Differding JT, Gardner RE, Roe BB: Intracardiac myxomas with report of two unusual cases and successful removal. Circulation 23:929, 1961. Balk AHM, Wagenaar SS, Bruschke AVG: Bilateral cardia myxomas and peripheral myxomas in a patient with recent myocardial infarction. Am J Cardiol 44:767, 1979.
Myocardial deposition
Communications
1295
Systemic light chain deposition is a rare finding in patients with plasma cell dyscrasias. Light chains can produce renal tubular damage and occasional glomerular abnormalities.‘*2 but except for certain forms of amyloidosis, widespread deposition of light chains is uncommon.3, 4 This case report describes systemic light chain deposition and associated myocardial coagulative necrosis without morphologic evidence of amyloidosis. Myocardial cell death has not previously been associated with plasma cell dyscrasias and systemic light chain deposition. A 35-year-old woman was referred to Hahnemann University Hospital with a 4-month history of weakness and fatigue. A workup begun at an outside hospital had revealed renal failure, acidosis, and an elevated serum calcium level. The patient had been started on hemodialysis and an elevated serum parathyroid hormone suggested primary hyperparathyroidism. Three and one-half parathyroids were surgically excised and were described as being hyperplastic on pathologic examination. Nonetheless, serum calcium levels remained elevated after parathyroidectomy. Physical examination revealed that the patient was disoriented as to time and place. The blood pressure was 100/60 mm Hg, the pulse was 98 bpm, the temperature was 100.6’ F, and the respirations were 22lmin. A right central facial palsy was present, and the remainder of the physical examination was unremarkable.
necrosis in light chain
Eric Staros, M.D., and Sheila Moriber Katz, M.D. Philadelphia,
Pa.
From the Department of Pathology University School of Medicine.
Reprint mann
requests: University,
and Laboratory
Medicine,
Hahnemann
Sheila Moriber Katz, M.D., Dept. of Pathology, HahneBroad & Vine Streets, Philadelphia, PA 19102.
Fig. 1. Electron micrograph of myocardium. Dense deposits are seen along the myocardial cell basement membrane and in the interstitial space (arrows). (Lead citrate, uranyl acetate; original magnification ~23,000.)
1296
December, 1985 American Heart Journal
Brief Communications
Fig. 2. The upper frame is a hematoxylin and eosin preparation which shows coagulative necrosis and cellular loss. The remaining interstitial network is thickened (original magnification x350). The lower frame shows kappa light chain fluorescent staining of the interstitium (arrow).
Additional studies included a normal chest x-ray examination, a calcium level of 13.5 mg/dl (normal greater than 9.2 and less than 11.0 mg/dl), and elevated liver enzymes as follows: serum glutamic oxaloacetic transaminase @GOT) 56 U/L (normal <34), alkaline phosphatase 372 (U/L (normal <74), and lactate dehydrogenase (LDH) 299 U/L (normal <196). The serum creatinine was 3.8 mg/dl (normal
cardiopathy. Despite the use of pressor agents, the patient became increasingly hypotensive and died. At autopsy, the heart weighed 520 gm and had a rubbery consistency. The coronary arteries were patent. Over visceral organs, most notably the lungs, spleen, kidneys and liver, were heavy and exhibited a similar firm consistency. On microscopic examination, the heart showed extensive regions of transmural myocytolysis, moderately thickened intramural coronary arteries, and the presence of homogeneous, eosinophilic interstitial tissue. Electron microscopy showed intramembranous dense deposits along myocardial and endothelial basement membranes. Immunofluorescent staining of the heart for kappa light chains was positive, while control sera for lambda light chain, fibrin, and complement compounds, were negative. Examination of the liver, pericardium, kidney, and breast showed intramembranous dense deposits along endothelial basement membranes by electron microscopic examination. The kidneys contained occasional glomeruli showing focal nodular sclerosis and multinucleated tubular epithelial cells, findings which are specific for light chain disease. Congo red stains for the detection of amyloid were negative and no fibrillar deposits were seen by electron microscopy. The bone marrow contained a moderately differentiated plasma cell infiltrate which stained for kappa chains by immunoperoxidase techniques. Dense deposits which are fluorescent when stained with fluorescein conjugated anti-light chain antisera are diagnostic for light chain deposition.5 While renal tubular damage and nodular sclerosis of glomeruli have been well described in light chain disease, systemic deposition is rare. Though mild congestive heart failure and nonspecific ECG changes have been described in association with systemic light chain deposition,5 the histopathologic findings of myocardial light chain disease have not been previously described. This case demonstrates an association between light chain disease and myocardial necrosis6 Because the patent coronary arteries exclude atherosclerosis as a cause of myocardial ischemia, necrosis appears to be a consequence of a cellular injury which was presumably caused by light chain deposition in intramural coronary arteries and the myocardial interstitium. REFERENCES
Bryan CW, Healy JK: Acute renal failure in multiple myeloma. Am J Med 44:128, 1968. 2. Levi DF, Williams RC Jr, Lindstrom FD: Immunofluorescent 1.
3.
4.
5.
6.
studies of the myeloma kidney with special references to light chain disease. Am J Med 44:922, 1968. Glenner GG, Ein D, Eanes ED, Bladen HA, Teny W, Page DL: Creation of “amyloid” fibrils from Bence Jones proteins in vitro. Science 174:712, 1971. Shirahama T, Benson MD, Cohen AS, Tamaka A: Fibrillar assemblage of variable segments of immunoglobulin light chains. J Immunol 110:21, 1973. Randall RE, Williamson WC, Mullinax F, Tung MY, Still WJS: Manifestations of systemic light chain deposition. Am J Med 60:29X 1976. Baroldi G: Different types of myocardial necrosis in coronary heart disease: Pathophysiologic review of functional significance. AM HEART J 89:742, 1975.