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MD,
Darla K. Liles,
MD,
MD
Case Report
An immunocompetent, 76-year-old black woman
presented to the emergency department of a tertiary care
facility with a 1-week history of intermittent dizziness,
lightheadedness, and diaphoresis. She denied any other
associated symptoms of shortness of breath, chest pain,
orthopnea, paroxysmal nocturnal dyspnea, palpitations,
or pedal edema. Her only other medical problem was
glaucoma. Upon admission, the only significant finding
at physical examination was an irregular heartbeat, with
a heart rate of 100 beats/min. An electrocardiogram (ECG)
was performed, which confirmed atrial flutter with variable
atrioventricular (AV) block with left-axis deviation. The
initial laboratory workup was unremarkable, showing
(continued next page)
Key Points
Primary cardiac lymphoma remains difficult to diagnose, and patient prognosis is poor.
The incidence of the disease is increasing, with immunocompromised patients and patients with acquired immunodeficiency syndrome accounting for most of the
increase.
Primary cardiac lymphoma should be considered in any
patient who has a cardiac mass and/or refractory pericardial effusion of undetermined cause, with the highest
suspicion raised in patients with a history of immunosuppression or acquired immunodeficiency syndrome.
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Fig. 1 Two-dimensional transesophageal echocardiogram in a modified 4-chamber view showing the right atrial mass in (A) systole
and (B) diastole. LA, left atrium; RA, right atrium.
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Case Report
cells were reactive with antibodies directed against leukocyte common antigen and had spotty reactivity with
L26 (CD20), which is a B-cell marker. Flow cytometry
revealed characteristics of large B-cell lymphoma of follicular center cell origin. The final diagnosis of the four
other specimens were a pericardial fibrotic lymph node,
a right atrial-wall fibrin clot, a left atrial-wall fibrin clot
that had a layer of malignant cells focally on the periphery similar to those in the right atrial mass, and pericardial fibrous and adipose tissue without malignancy.
Postoperatively, the patient required inotropic support for 4 days. She had intermittent episodes of AV
dissociation with first degree AV block. She was atrially
paced initially, with removal of the pacing wires on the
eighth postoperative day. She remained in sinus rhythm
from that point on until she was discharged 3 days later.
Radionuclide angiography was performed as part of the
baseline protocol in preparation for chemotherapy and revealed normal biventricular systolic function with a left
ventricular ejection fraction of 66%. A long-term, indwelling, right upper central line was placed for chemotherapy
infusion. She was treated with the CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy regimen and tolerated it well initially. Soon after completing her second cycle of CHOP, however, she developed
neutropenic fevers. Despite receiving appropriate supportive care, she died 10 weeks after surgery due to sepsis
related to severe immunosuppression from chemotherapy.
Discussion
PCL has been identified predominantly as a B-cell lymphoma of diffuse large cell type,1 and it seems to have a
propensity to involve the right chambers of the heart.3 PCL is
extremely rare in immunocompetent patients, accounting for
1.3% of all cardiac tumors and 0.5% of all extranodal1 lymphomas.28 The incidence of the disease is increasing, primarily in immunocompromised patients, and patients with acquired immunodeficiency syndrome (AIDS) are most at
risk.3,28 It has a high mortality rate due to the advanced stage
of myocardial involvement at initial presentation.
Clinical presentation is variable, with a wide array of
symptoms that could be cardiac or noncardiac, leading to
difficulty in recognizing cardiac lymphoma.11,29 35 The initial workup should include chest x-ray, TTE, and CT. A
review of 48 published cases of PCL between 1980 and 1996
revealed that chest x-ray, TTE, and CT are standard procedures in the workup of this disease, but TEE and magnetic
resonance imaging had greater than 90% sensitivity and could
provide more information that would allow for early confirmation of suspected PCL.3 Pericardiocentesis can provide a
diagnostic cytology specimen if pericardial effusion is
Southern Medical Journal Volume 96, Number 8, August 2003
Conclusions
PCL remains difficult to diagnosis, and the prognosis is
poor. Because of the rarity of the disease itself, the variability
of the clinical manifestations, the limited noninvasive diagnostic techniques available, and the difficulties and/or delays
in the use of invasive measures, the diagnosis of PCL is
extremely difficult to make. Fewer than 100 cases have been
reported in immunocompetent patients. The incidence of the
disease is increasing, with immunocompromised and AIDS
patients accounting for the highest number of increased reports. PCL should be considered in any patient who has a
cardiac mass and/or refractory pericardial effusion of undetermined etiology, with the highest suspicion in patients with
a history of immunosuppression or AIDS.
Acknowledgment
We thank the members of the Cardiothoracic Surgery
Division of the Brody School of Medicine for their assistance
and for the expert care that they provided in the management
of this patient.
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