Please select Print from the file menu or right mouse click and choose print from the menu to print this page.

[13] Sudden Cardiac Death in Non-Cardiac Primary Hematolymphoid Malignancy

KM Jones, EN Pavlikso, LR DiBernardo. Duke University Medical Center, Durham, NC

Background: Cardiac involvement by lymphoid or leukemia may be underdiagnosed and directly responsible for significant morbidity and mortality. We describe five patients with non-cardiac primary hematolymphoid malignancy who experienced sudden cardiac death. All had extensive cardiac involvement by tumor identified at autopsy, clinically unrecognized in four of the cases.
Design: Autopsy was performed in four cases with en bloc removal of the organs, dissection of the heart and evaluation of the coronary arteries and conduction system. One case was limited to solid organ biopsies through a small posterior incision. Routine histochemical staining was performed using standard methods.
Results:

Case	Age (years)	Tumor	Clinical Findings	Autopsy Cardiac Findings
1	59	Burkitt lymphoma	Restrictive cardiomyopathy; bradyarrythmia	Extensive interstitial and epicardial lymphoma, also involving sino-atrial node
2	60	Burkitt-like high grade lymphoma	Syncope	Extensive interstitial and epicardial lymphoma compressing the left circumflex artery resulting in 75% luminal stenosis
3	44	Acute myeloid leukemia	Chest pain; tachycardia	Extensive interstitial and epicardial leukemic infiltrate; fibrinous epicarditis; right atrial and left ventricular hemorrhages
4	78	Diffuse large B-cell lymphoma (DLBL)	Dyspnea; hypotension; pericardial effusion	Intravascular lymphoma with interstitial infiltrate and myocardial infarction
5	73	DLBL	Chest pain; mediastinal and cardiac mass on MRI	Extensive interstitial lymphoma with rupture of ventricular free wall

Conclusions: The majority of cardiac tumors are metastatic, of which hematolymphoid metastases are frequent and may lead to sudden cardiac death by different mechanisms as illustrated in this case series. Patient 1 had a restrictive cardiomyopathy and refractory bradyarrhythmia with lymphoma involving the sinoatrial node. Patient 2 had syncope with severe coronary artery compression associated with epicardial and interstitial lymphoma. Patient 3 had features of diastolic dysfunction and tamponade with an epicardial leukemic infiltrate. Patient 4 had intravascular and interstitial lymphoma with myocardial infarction, and patient 5 had interstitial lymphoma with ventricular rupture, illustrating the variable distribution and pathophysiology of DLBL involving the heart. Importantly, cardiac involvement by tumor was unrecognized until the autopsy for patients 1 through 4, reflecting a clinical diagnostic gap that may be improved with greater awareness and earlier recognition of cardiac involvement in patients with lymphoma or leukemia.
Category: Autopsy

Monday, March 3, 2008

Poster # 9, Monday Morning