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(Circulation. 1998;97:220-221.)
© 1998 American Heart Association, Inc.

Images in Cardiovascular Medicine

Primary Cardiac B-Cell Lymphoma

Hiroshi Tada, MD; Ken-ya Asazuma, MD; Eichi Ohya, MD; Takio Hayashi, MD; Tsuguhiko Nakai, MD; Takashi Nakayama, MD; ; Takanori Ueda, MD

From the Third (H.T., K.A., T.H., T. Nakai) and First (T. Nakayama, T.U.) Departments of Internal Medicine, Fukui Medical School, and the Department of Internal Medicine (E.O.), Izumigaoka Onsen Hospital, Fukui, Japan.

A 67-year-old woman was admitted to a hospital because of the recent onset of general malaise. She had a classic lilac-colored rash over her eyelids, the bridge of her nose, her cheeks, elbows, and knees and weakness in the proximal limb muscles. A diagnosis of dermatomyositis was confirmed by skeletal muscle biopsy. She was started on a course of oral glucocorticoids. Three months later, she complained of dyspnea. An echocardiogram revealed a massive pericardial effusion with evidence of both right atrial and ventricular collapse consistent with cardiac tamponade. Pericardiocentesis yielded 1000 mL of exudative bloody fluid with a lactate dehydrogenase value of 23 950 IU/L. Cytology revealed cells believed to represent lymphoma.

The patient was referred to Fukui Medical School for further investigation in August 1995. Transesophageal echocardiography showed a dense thick mass in the right atrioventricular groove extending into the right ventricular free wall (Fig 1A). The right ventricular wall and the left ventricular inferior wall were thickened and exhibited high echogenicities (Fig 1B). A small pericardial effusion was also noted. MRI demonstrated a thick mass in the right atrioventricular groove and the thickened right ventricular and left ventricular inferior walls, suggesting significant massive cardiac involvement (Fig 2A through 2C). The brightness of these lesions and pericardium was increased in T2-weighted images, and Gd-DTPA enhanced the lesions heterogeneously (Fig 2D). Contrast right atrial angiography demonstrated a zonal filling defect at the right atrioventricular groove, indicating cardiac involvement in that region (Fig 3). Full-body CT and MRI showed no abnormal lymph node swelling. ⁶⁷Ga scintigraphy revealed significant abnormal uptake in the heart (Fig 4A). Pericardiocentesis was performed a second time to establish a definite diagnosis. Cytological study showed cells consistent with large-cell lymphoma (Fig 4B). Surface markers were positive for cluster of differentiation (CD) 19, CD22, CD45, and HLA-DR (human leukocyte antigen-D–related) sIgM and s, indicating that the cells were of B-cell origin. Thus, the patient was diagnosed with primary cardiac B-cell lymphoma with massive cardiac involvement. She is currently undergoing medical treatment with chemotherapeutic agents.

View larger version (99K): [in this window] [in a new window]   Figure 1. Transesophageal echocardiographic images. A, Dense, thick mass is visible in right atrioventricular groove with extension into right ventricular free wall. B, Right ventricular wall is thickened and exhibits high echogenicity. A small pericardial effusion is present. RA indicates right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; and Ao, ascending aorta.

View larger version (148K): [in this window] [in a new window]   Figure 2. ECG-gated, T1-weighted MRIs. A, Transverse image through middle of right atrium showing thick mass in right atrioventricular groove. B, Saggital image showing thickened right ventricular wall. C and D, Saggital images differing from B. Brightness of these lesions and pericardium are increased on T2-weighted images, and Gd-DTPA enhances them heterogeneously (D). PA indicates pulmonary artery. Other abbreviations as in Fig 1.

View larger version (129K): [in this window] [in a new window]   Figure 3. Right atrial angiogram in right anterior oblique projection. Massive involvement in right atrioventricular groove appears as zonal filling defect. Abbreviations as in previous Figures.

View larger version (79K): [in this window] [in a new window]   Figure 4. A, 67Ga scintigraphic image. Intense 67Ga uptake is visible in heart. B, Pericardial fluid obtained from patient's second pericardiocentesis, showing malignant cells with features of large-cell type of malignant lymphoma (magnification x1000).

Footnotes

Reprint requests to Hiroshi Tada, MD, the Third Department of Internal Medicine, Fukui Medical School, 23 Shimoaizuki, Matsuoka-cho, Fukui 910-11, Japan.

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to Dr Hugh McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC1-267, Houston, TX 77030.

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