View source for Lymphoma case study one

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{{Lymphoma}}
{{CMG}}; '''Associate Editor-In Chief:''' Apranta Deka Patel, M.B.B.S, M.D.

==Case #1- Primary Cardiac Lymphoma- Rare EBV-negative Post-transplant Lymphoproliferative Disorder==

===Case Presentation===

A 77-year-old woman with past medical history of [[hypertension]], [[diabetes mellitus]] and [[autoimmune hepatitis]] status-post [[liver transplant]] presented to the [[Emergency Department]] for symptoms of worsening [[dyspnea on exertion]] progressing to [[shortness of breath]] at rest and [[pleuritic chest pain]] over the course of three months. Review of systems was otherwise negative.

Her home medications included [[tacrolimus]], [[mycophenolate]], [[amlodipine]], [[valsartan]], [[metoprolol]], [[atorvastatin]], [[metformin]], [[glipizide]] and [[levothyroxine]].

===Past Medical History===

Other than what is mentioned above, her [[autoimmune hepatitis]] was complicated by liver [[cirrhosis]] for which she had an orthotopic [[liver transplant]] four years prior to this presentation. [[Hepatocellular carcinoma]] and [[mixed cholangiocarcinoma]] were found on the explant with no evidence of spread. Post-transplant course was otherwise uncomplicated on stable doses of [[immunosuppresants]], [[tacrolimus]] and [[mycophenolate]].

===Physical Examination===

Vitals were stable with [[blood pressure]] of 155/100 mmHg, [[heart rate]] of 87 /min, mild [[tachypnea]] and [[oxygen saturation]] of 98% on room air. No [[pallor]], [[icterus]] or palpable [[lymph nodes]] were found on exam. Lung, abdominal and neurologic exams were normal. No [[pedal edema]] or [[jugular venous distension]] was noted. Heart sounds [[S1]] and [[S2]] were normal without any [[murmurs]], [[rubs]] or [[gallops]].

===Laboratory and Imaging Findings===

Initial laboratory evaluation revealed normal values for [[complete blood count]], [[liver function tests]] and renal function panel but showed elevated [[D-dimer]] level at 1445 mg/mL (ref range 0-500 ng/mL).

[[Computed tomography angiography]] was performed which ruled out [[pulmonary embolism]] but revealed a cardiac mass indenting the right heart chambers, extending [[inferiorly]] from the region of the [[atrioventricular groove]] along the right base of the heart measuring approximately 11x 7x 5 cm. This mass was noted to be encasing the [[right coronary artery]].

<gallery>
Image:CTA_sagittal_PTLD_RCA.jpg‎ 

Image:CTA_coronal_section_PTLD.jpg‎

Image:CTA_cross_sectionPTLD.jpg‎

</gallery>
[[Image:Subcostal_TTE.jpg‎|Transthoracic echocardiography subcostal view showing the cardiac mass adjacent to and compressing the right atrium|left|300x400px]]

[[Transthoracic echocardiogram]] also demonstrated a large [[hypoechoic]] mass likely external to the [[right atrium]] arising from the region of the [[atrioventricular groove]] and compressing the [[right atrium]]. The [[left ventricular ejection fraction]] was normal. There was a loculated [[pericardial effusion]] measuring 3cm adjacent to the [[right ventricle]] with no clear signs of [[tamponade]].








[[Cardiac magnetic resonance imaging]] was performed which stated a similar size with more details of the extent of the mass which was noted to invade the right [[atrial]] as well and right and left [[ventricular apical myocardium]].

[[Image:CardiacMRI_massPTLD.jpg‎|Cardiac MRI showing cardiac mass invading right atrial and right and 
left ventricular apical myocardium with compression of right atrial 
chamber|left|200x300px]]

===Diagnosis and Management===

A [[sternotomy]] was performed with the plan for debulking and procuring tissue for [[biopsy]]. However, during induction of anesthesia, this was complicated by tamponade physiology needing emergent [[pericardiotomy]] which yielded [[serosanguinous]] fluid. Attempt at debulking failed due to friable cardiac tissue but tissue for pathology was obtained.

[[Image:Monomorphic_high_grade_PTLD.jpg‎|Pathology:Monomorphic high grade B cell PTLD|left|200x300px]]



Biopsy of cardiac mass revealed monomorphic PTLD or [[Post-transplant lymphoproliferative disorder]] of [[high grade large B-cell lymphoma]] type. Pending EBV ([[Epstein-Barr virus]]) status test results, the patient was initiated on [[rituximab]] with the plan to follow it up with [[CHOP]]21 ([[cyclophosphamide]], [[doxorubicin]], [[vincristine]] and [[prednisolone]]) for four cycles with the presumption of EBV positivity.


However, the blood [[EBV]] [[PCR]] and [[EBER ISH]] (EBV-encoded, nonpolyadenylated RNAs) tests came back negative and rituximab was stopped. Modified [[CHOP]] regimen was started and is currently ongoing pending tumor response.



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