The risk of NHL is 60 times higher in patients with human immunodeficiency virus (HIV) infection than in the general population.³ When compared with NHL in the general population, most HIV-associated NHLs are high-or intermediate-grade B-cell malignancies, but T-cell lymphomas have also been reported with increased frequency. Approximately 85% of HIV-associated NHLs have extranodal involvement, often in unusual sites, such as the orbit, heart, muscles, adrenal glands, skin, and salivary glands.³ When compared with other types of lymphoma, T-cell lymphoma may involve the heart and great vessels with increased frequency. The mechanism of this positive cardiac tropism is uncertain, but it might be related to an increased propensity of T-cell lymphoma to metastasize in a hematogenous pattern.

His recent medical history was notable for stage IVB peripheral T-cell lymphoma diagnosed 4 months earlier. At diagnosis, staging had shown widespread supra- and infradiaphragmatic nodal involvement as well as multiple extranodal involvement including muscles, bilateral adrenal glands, and subcutaneous nodules, but not bone marrow. The patient had achieved a complete response to induction chemotherapy consisting of GND (Gemcitabine, Navelbine, and Doxil) and of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) augmented by high-dose methotrexate. He remained in remission for 2 weeks, at which time he developed isolated central-nervous-system relapse presenting as right-arm weakness. He was found to have a 2.1-cm homogeneously enhancing mass in the left motor cortex consistent with recurrent lymphoma, for which he underwent whole-brain irradiation. Immediately after the completion of brain irradiation, he developed recurrent lymphoma systemically, including bilateral adrenal masses and possibly invasion of the kidneys.

His earlier medical history was notable for HIV diagnosed more than 25 years earlier and for a non-ST-elevation myocardial infarction in association with coronary artery disease, which had been treated 3 years earlier via placement of a bare-metal stent in the left anterior descending coronary artery.

On examination, the patient was dyspneic at rest, needing to stop mid-sentence when talking, and he was using accessory muscles of respiration. His blood pressure was 106/51 mmHg, his heart rate was 81 beats/min, and there was no pulsus paradoxus. The jugular venous pulsation was elevated at 10 cm but without Kussmaul's sign. Cardiac auscultation was notable for an old 2/6 systolic ejection murmur at the base and an S₄ heart sound. Crackles were present bilaterally at the lung bases. There were no subcutaneous nodules, hepatosplenomegaly, or lymphadenopathy. A chest radiograph was consistent with pulmonary edema, and an electrocardiogram (ECG) showed evidence of a prior anteroseptal myocardial infarct, with more prominent ST elevation in leads V₁ through V₃ than had been seen on an ECG 3 weeks earlier. The troponin I level was elevated at 0.16 ng/mL, the total creatine kinase level was 157 U/L, and the creatine kinase-MB level was 30 ng/mL.

On the basis of these findings, an acute coronary syndrome was strongly suspected. The patient was started on aspirin and on intravenous heparin and eptifibatide, with plans for cardiac catheterization. An echocardiogram showed a left ventricular (LV) ejection fraction of 0.65 to 0.70, with apical hypokinesis and a hyperdynamic mid ventricle. There was a small, free-flowing pericardial effusion without echocardiographic evidence of tamponade. On repeat testing immediately before cardiac catheterization and approximately 12 hours after the initiation of heparin and eptifibatide infusions, the patient's platelet count was noted to have decreased from 106 to 38 × 10⁹/L, without any change in white blood cell count or hematocrit level. The eptifibatide and heparin were discontinued and plans for catheterization were deferred, given (especially) the resolution of all symptoms with medical therapy. An evaluation—including measurement of the patient's anti-platelet factor 4 antibodies, lactate dehydrogenase, and haptoglobin—was begun to look for the cause of the patient's new thrombocytopenia.

The patient remained stable without any change in his vital signs over the next 6 hours but then experienced abrupt onset of chest pain and worsened shortness of breath that evening. As the patient was being examined, he suddenly became pulseless. Telemetry monitoring revealed a new right bundle branch block with a prolonged PR interval followed by abrupt complete heart block. The patient regained a pulse after immediate cardiopulmonary resuscitation (CPR) followed by the administration of 1 mg of epinephrine and atropine, but within 10 minutes he again went into asystole, requiring re-initiation of CPR. He was started on an epinephrine drip and transcutaneous pacing. A secondary survey revealed absent breath sounds over the left hemithorax. Needle thoracostomy released more than 4 L of blood from the patient's chest. The patient again lost a pulse. Bilateral femoral central venous lines were placed, with infusion of more than 12 L of crystalloid. Despite continued CPR, fluid resuscitation, and multiple rounds of vasopressor agents, the patient did not regain a pulse and was pronounced dead.

Consent was obtained for an autopsy. Gross findings revealed widespread extranodal lymphoma involving the kidneys, adrenal glands, thyroid, psoas muscle, lungs, bowel, brain, and heart. There was no involvement of the bone marrow or the meninges. Examination of the heart showed the pericardial sac to be markedly thickened by extensive tumor (Fig. 1A). There was a transmural free-wall rupture of the anterolateral left ventricle, remote from an anteroseptal scar from prior myocardial infarction (Fig. 1B), with a pericardial effusion of approximately 30 cc of blood and clots. There was no obstructive epicardial coronary artery thrombus, and the patient's stent was patent. Microscopic examination of the heart disclosed extensive and diffuse tumor infiltrates and, in the lateral LV free wall, sheets of malignant lymphocytes along the site of rupture (Fig. 2). No acute myocardial infarct was present.

On microscopic examination, the lymphoma cells appeared to lack cohesion, which quite possibly predisposed the heart to free-wall rupture in the setting of thrombocytopenia. Once the LV ruptured, cardiac tamponade ensued abruptly, despite the presence of only 30 cc of blood in the pericardial space, due to a poorly compliant pericardium that was also heavily infiltrated with tumor. No direct connection could be found between the pericardial sac and the pleural space; it is unclear whether the free-wall rupture tracked into the pleural space, or whether the massive hemothorax resulted from resuscitation-related bleeding in the setting of thrombocytopenia.

To our knowledge, this is the 1st reported case of LV free-wall rupture due to transmural infiltration of HIV-associated peripheral T-cell lymphoma. Other authors have reported right ventricular rupture from a likely T-cell lymphoma,⁶ T-cell lymphoma metastatic to the right ventricle that was responsive to chemotherapy,⁷ and natural killer/T-cell lymphoma directly invading the left atrium and interventricular septum.⁸ Although many diagnoses are made only at autopsy, diagnosis of cardiac infiltration by lymphoma has in some cases been made by means of cardiac magnetic resonance imaging⁷ or endomyocardial biopsy.⁹