HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

History
60-year-old male, with no known drug allergies and no family history of interest. Personal history of:
Cardiovascular risk factors (CVRF): obesity, arterial hypertension and ex-smoker Surgical intervention of vasectomy complicated with epididymitis 32 years ago
On treatment with amlodipine 5 mg at breakfast.

Current illness
He presented with 5 months of progressive exertional dyspnoea with frank worsening in the last month to NYHA class III, associated with oedema of the lower extremities up to the abdominal wall, orthopnoea and decreased diuresis, for which he attended the emergency department.
In the initial study in the ED, after finding elevated D-dimer and signs of right heart failure, it was decided to perform a thoracic CT angiography, initially ruling out pulmonary thromboembolism but revealing thickening and moderate pericardial effusion. Hospital admission was decided.

Physical examination
Blood pressure (BP) 130/80 mmHg. Heart rate (HR) 128 bpm, profuse diaphoresis, sensation of gravity, although good distal perfusion. Head and neck: jugular ingurgitation with hepatojugular reflux. Cardiac auscultation: irregular and muffled sounds. Pulmonary auscultation: global hypophonesis with crackles in the right base. Abdomen: distended, wall oedema with fovea. Lower limbs: oedema with fovea up to the root of the thighs.

COMPLEMENTARY TESTS
12-lead surface electrocardiogram (ECG): atrial fibrillation at 115 bpm with decreased QRS amplitude. EV isolated. The corrected QT interval is normal.
Chest X-ray: right costophrenic sinus impingement and marked cardiomegaly.
Laboratory tests (in the ED): NT-proBNP of 114 pg/ml. Haemogram, renal function parameters and ionogram in normal ranges. Coagulation normal except for elevated D-dimer: 1100 mg/dl.
CBC (hospitalisation): normal haemogram, ESR 51 mm/h. Normal blood biochemistry, normal transaminases and ions. Normal iron profile
Immunoglobulins: IgG 1170 mg/dl (768-1632), IgA 395 mg/dl (68-378), IgM 115 mg/dl (60-263). Beta 2 microglobulin: 3.72 mcg/ml (1.9-2.53). Interferon gamma-TBC: negative.
Serology: HIV, HBV, HCV, Lues, EBV, HSV: negative.
Pathological anatomy of pericardial fluid: positive cytology for malignancy.
Immunohistochemical study: CD79a+, CD3+ in accompanying cellularity, PAX5+, CD20+, Bcl2+, Bcl6-, MUM1+, C-Myc weakly positive in 40% of cells, CD10-, CD30-, EBER-, CD138-, CD38-, HHV8: negative. Ki 67: 80%. Compatible with diffuse large B-cell lymphoma.
Flow cytometry of pericardial fluid: in the sample analysed 77% of lymphocytes were observed, 95% of which correspond to an apparently mature B population (CD20+, FMC7+, CD10-) and without intracytoplasmic TdT, suggesting a B lymphoproliferative process with a non-specific phenotype.
Pericardial fluid culture: no development of microorganisms.
Pathological anatomy of the iliac crest (bone biopsy): normocellular bone marrow, no infiltration by lymphoma.
Chest CT scan: subpleural pseudonodule 10 mm in diameter located in the posterior segment of the right upper lobe to be monitored. Moderate pericardial effusion with wall thickening. Cardiac cavities within normal. Ascitic fluid. Dorsal spondylosis.
PET-CT: imaging shows no significant hypermetabolism in the thickened pericardium. Small left laterocervical, supraclavicular lymph nodes with SUV max 1.33, infracentimetric and an analogous one on the right with SUV max 1.9. In the mediastinum small lymph nodes with SUV max 1.57, the largest 12.8 mm and highest metabolic activity SUV max 2.5. Retroperitoneal small crural, pericellular and para-aortic nodes with low metabolic SUV 1.08. Hypermetabolic lesion (SUV max 9) hyperdense and irregular morphology in left testicle highly suggestive of malignancy.
Testicular ultrasound: image of a 10 mm spermatocele in the head of the left epididymis and changes secondary to left vasectomy.
Left testicular pathological anatomy: left orchiectomy specimen: no evidence of neoplasia. Acute and chronic epididymitis associated with fibrous and histiocytic reaction. Testicular parenchyma and spermatic cord without histological alterations.
Transthoracic echocardiogram: preserved left ventricular systolic function (52%), moderate biauricular dilatation and severe pericardial effusion with evidence of visceral pericardial thickening without cavity collapse or respiratory variations of valvular flows.
Control transthoracic echocardiogram: LVEF 61%, mild pericardial effusion, without clear evidence of constrictive physiology.

CLINICAL EVOLUTION
The patient was admitted for study and treatment with the combined participation of the cardiology, internal medicine and haematology departments. Initially, the patient showed little response to depletive treatment, with persistence of marked signs of right heart failure and systolic blood pressure around 90-100 mmHg, although without clear evidence of haemodynamic compromise. He maintained a good response to atrioventricular (AV) node braking therapy and anticoagulation by CHASVASC:2 and HASBLED:1 with rivaroxaban was initiated. As part of the complementary study, transthoracic echocardiography showed severe pericardial effusion without significant repercussions on the right chambers, and a diagnostic-therapeutic pericardiocentesis was considered. This technique yielded 950 cc of serohaematic fluid with a clear immediate clinical improvement and samples were sent to microbiology and pathological anatomy, for filiation of the effusion, which finally demonstrated the presence of blast cells compatible with diffuse large B-cell lymphoma, with no infectious microbiological evidence.
The study was completed with a PET-CT scan showing lymphadenopathy with slight increased metabolism and a hypercapillary lesion in the left testicle (image 4) with metabolic criteria of malignancy, and we requested a testicular ultrasound scan which did not reveal a clear malignant testicular mass (10 mm spermatocele in the head of the left epididymis and changes secondary to vasectomy).
Given that the origin of the malignant process had not been identified and the patient was not in a suitable clinical condition to take a biopsy of the pericardium or mediastinal lymph nodes by thoracoscopy, the case was assessed by the haematological tumour committee and given the intense PET-CT uptake in the left testicle, a diagnostic orchiectomy was decided for assessment of malignancy at this level, given the prognostic and therapeutic implications of testicular involvement by high-grade lymphoma. With the diagnosis of high-grade B lymphoma in the pericardium, it was decided not to delay chemotherapy treatment with R-CHOP/21, administering the first cycle (with Truxima) and with no immediate adverse reactions.
We performed a control transthoracic echocardiogram with mild pericardial effusion and increased LVEF (61%). Oedema resolved and he remained cardiologically stable. There were no complications with haematological treatment, so it was decided to discharge him and continue his treatment on an outpatient basis. Weeks later, the result of the testicular biopsy was negative for malignancy.

DIAGNOSIS
Effusivoconstrictive pericarditis.
Atrial fibrillation with rapid mid ventricular response first episode. Congestive heart failure with preserved LVEF.
Stage IV diffuse large B-cell lymphoma.
