Ann Thorac Surg 2002;73:1310-1312
© 2002 The Society of Thoracic Surgeons
Case report
Cardiac involvement in a case of acute lymphoblastic leukemia
Ashutosh Hardikar, MCh, FRCS*a,
Prem Shekar, MCh, FRCSa,
John Stubberfield, FRACSa,
David R. Craddock, FRCS, FRACSa,
Leon P. Bignold, FRCPAb
a Department of Cardiothoracic Surgery, The Royal Adelaide Hospital Adelaide, Australia
b Division of Tissue Pathology, Institute of Medical and Veterinary Science, Adelaide, Australia
Accepted for publication August 7, 2001.
* Address reprint requests to Dr Hardikar, Department of Cardiothoracic Surgery, Royal Adelaide Hospital, Level IV, East Wing, North Terrace, Adelaide 5000, South Australia
e-mail: a_hardikar{at}hotmail.com
 |
Abstract
|
|---|
We present an extremely rare case of an immunocompromised patient with a T-cell acute lymphocytic leukemia relapse presenting as a right atrial tumor. Problems in diagnosis, vulnerability due to previous immunosuppression and bone marrow transplant, and successful surgical excision are highlighted. Cardiac involvement with hematologic neoplasms should be taken with more than academic interest, as it may be amenable to treatment.
|
Introduction
|
|---|
Cardiac infiltration by hematologic neoplasms leading to clinically detectable cardiovascular disease is rare. Such manifestations are likely to be more common as these neoplasms are more amenable to therapy than heretofore, and the proportion of immunocompromised population is on the rise [1–4]. Extranodal malignant leukemias or lymphomas involving only the heart without dissemination is extremely rare and very few antemortem cases have been reported. We report an immunocompromised patient with a T-cell acute lymphocytic leukemia (ALL) relapse presenting as a right atrial tumor.
A 31-year-old Caucasian man presented in September 1998 with shotty lymph node enlargements in the neck and raised white cell count with 86% blast cells. Investigations showed him to have ALL, the L2 subtype. Seventy-six percent of the blasts in the bone marrow showed 11 q 23 rearrangement. Blood tests were negative for human T-cell lymphoma virus, human immunodeficiency virus, or the hepatitis group of viruses. He was treated with leukopheresis and chemotherapy (LaLa [Leucemie Aigue lymphoblastiquede l’Adulte]), to which he responded poorly. Heavy contamination of the CD34+ fraction of his autologous stem cells by leukemic cells precluded autologous bone marrow transplant (BMT). Hence, matched unrelated donor BMT was performed in December 1998. Post-BMT, the immunosuppression consisted of cyclophosphomide, total body irradiation, and antithymocyte globulin. The postoperative period was complicated by severe mucositis, strepto and enterococcus bacteremia, epistaxis, mild clinical veno-occlusive disease, conjugated hyperbilirubinemia, depression, transient mental confusion, and grade II skin graft versus host disease which responded to intravenous steroids. He later suffered from an episode of left lower lobe pneumonia in 1999.
In September 2000, he presented again with swollen abdomen and ankles. He was essentially afebrile and diagnosed to have ascites, with the possibilities of recurrent ALL and inferior vena caval thrombosis in mind. He was investigated with a computed tomographic abdomen and duplex study for the great vessels, which failed to pinpoint anything other than the ascites. Finally, he underwent a diagnostic laparotomy when 1.5 liters of ascitic fluid was found with white cell count of 23,000. No organisms were grown on repeated cultures. He was then labeled as a case of spontaneous bacterial peritonitis and the ankle swelling was attributed to isolated right heart failure, for which an echocardiogram was performed. This showed him to have a large right atrial mass involving interatrial septum and partially obstructing the tricuspid orifice.
He was then taken up for surgery on a semi-urgent basis. At surgery, the right atrium was found to be greatly distended due to an intraluminal mass. The pericardium was grossly not involved. Venous cannulation was established using direct superior vena caval and inferior vena caval cannulation. Right atriotomy revealed a 12 x 11 x 3-cm mass, which was excised along with its attachment to the interatrial septum. The mass weighed 98 gm (Fig 1).
Tricuspid valve was intact and the rest of the heart had no focal macroscopic involvement.
Histologic evaluation of the mass showed it to be a uniform tumor composed of small cells having scant cytoplasm and vesicular nuclei (Fig 2).
The immunoperoxidase staining showed it to be consistent with T-cell acute ALL relapse. Flow cytometry showed the phenotype as CD1+ CD5+ CD7+ CD4- CD8- CD34+ HLA-DR-, CyCD3+. The margins were found to be containing malignant cells. The patient did well in the postoperative period, except episodes of supraventricular tachycardia, which responded to digoxin. He was discharged uneventfully on Warfarin (as the interatrial septum was denuded). He underwent a high resolution computed tomographic scan of the chest for pulmonary recurrences and a transthoracic echocardiogram, which ruled out focal macroscopic disease. Now at 3 months postoperatively, although he has no cardiac related symptoms, he has presented with testicular and skin recurrence. Because of this, and the fact that the margins of the cardiac mass were not free, he has been on intensive chemotherapy once again.
View larger version (155K):
[in this window]
[in a new window]
|
Fig 2. Histology of the atrial tumor showing closely packed lymphoid cells with occasional mitoses. (Hematoxylin and eosin; original magnification x400.)
|
|
|
Comment
|
|---|
Cardiac involvement by metastatic neoplasms is relatively uncommon and usually occurs with widely disseminated disease. Very few cases of hematologic malignancies have been reported, and most have been lymphomas or T-cell leukemia lymphoma [2, 3, 5, 6]. Even primary cardiac lymphomas have been described [2]. In a review of 3,314 consecutive autopsies, 806 (24.3%) had malignancies, and 95 (11.8%) of these had cardiac involvement [1]. The most common malignancies encountered in the order of decreasing frequency were lung, lymphoma, breast, leukemia, stomach, melanoma, liver, and colon. Cardiac involvement has been described in chronic adult T-cell leukemia. It is caused by human T-cell lymphoma virus-1 and is endemic in southwest Japan, the Caribbean, Africa, and South America [5]. We report this rare case of cardiac involvement in a relapse of ALL.
In a review of clinical and autopsy study of cardiac involvement in 45 bone marrow transplantation patients [4], 15 showed electrocardiogram ST segment or T-wave changes and arrhythmias. Fifteen patients had increased heart weight, with myocardial edema, fibrosis, and cellular hypertrophy predominating. Only 2 had marantic endocarditis of the aortic valve. They found that clinically significant heart involvement affects 5% to 10% of patients undergoing BMT after undergoing pretreatment with cyclophosphamide and total body irradiation. Acute T-cell leukemia/lymphoma involving the myocardium diffusely is known. A patient with double valve replacement for the same was described at autopsy as having widespread cardiac infiltration with malignant cells [3]. One hundred and thirty-one cases with pericardial involvement in advanced malignancy have been described which presented with features of tamponade [7]. Lung is the most common primary, followed by lymphomas and leukemias. In the present case, we see a very large (12 x 11 x 3 cm) involvement of the right atrium with no obvious involvement of myocardium, pericardium, or valvular tissue.
Right atrial tumors, commonly myxomas, presenting with features of right heart failure, with evidence of tricuspid stenosis have been described [8]. Isolated cardiac aspergillosis after bone marrow transplant has also been described [9]. In the present case, the mass went undiagnosed for a long time. He even underwent an exploratory laparotomy, when a timely echocardiogram would have revealed the diagnosis.
To conclude, we point out that cardiac involvement with hematologic neoplasms should be taken with more than academic interest, as it may be diagnosed early and may be amenable to aggressive treatment [6].
|
References
|
|---|
-
Abraham K.P., Reddy V., Gattuso P. Neoplasms metastatic to the heart: review of 3314 consecutive autopsies. Am J Cardiovasc Pathol 1990;3:195-198.[Medline]
-
Margolin D.A., Fabian V., Mintz U., Botham M.J. Primary cardiac lymphoma. Ann Thorac Surg 1996;61:1000-1001.[Abstract/Free Full Text]
-
Furihata M., Ido E., Iwata J., et al. Adult T cell leukemia/lymphoma with massive involvement of cardiac muscle and valves. Pathol Int 1998;48:221-224.[Medline]
-
Kupari M., Volin L., Suokas A., Timonen T., Hekali P., Ruutu T. Cardiac involvement in bone marrow transplantation: electrocardiographic changes, arrhythmias, heart failure and autopsy findings. Bone Marrow Transplant 1990;5:91-98.[Medline]
-
Takata J., Taguchi H., Miyoshi I., Doi Y.L. Cardiac valve invasion in chronic adult T cell leukemia. Heart 1998;80:311-312.[Free Full Text]
-
Wiernik P.H., Sutherland J.C., Stechmiller B.K., Wolff J. Clinically significant cardiac infiltration in acute leukemia, lymphocytic lymphoma, and plasma cell myeloma. Med Pediatr Oncol 1976;2:75-85.[Medline]
-
Islam N., Ahmedani M.Y. Renal carcinoma presenting as cardiac tamponade: a case report and review of literature. Int J Cardiol 1998;64:207-211.[Medline]
-
Frishman W., Factor S., Jordan A., et al. Right atrial myxoma: unusual clinical presentation and atypical glandular histology. Circulation 1979;59:1070-1075.[Abstract/Free Full Text]
-
Johnson R.B., Wing E.J., Miller T.R., Rosenfeld C.S. Isolated cardiac aspergillosis after bone marrow transplantation. Arch Intern Med 1987;147:1942-1943.[Medline]
This article has been cited by other articles:
|
|
|
|
 
J. P. Veinot
Endomyocardial biopsy of cardiac neoplastic involvement
Ann. Thorac. Surg.,
April 1, 2003;
75(4):
1363 - 1364.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
