Extramedullary disease in MM is a recognised manifestation of MM characterised by involvement of different anatomical sites distant from the bone marrow including lymph nodes, skin, breast, liver, central nervous system [5–13]. Pericardial involvement is particularly rare with myelomatous pericardial effusions present in less than 1% of all cases of MM . The first case reported in literature was in 1949 by Chorg . In our case, no symptoms of MM were found. The monoclonal spike discovered in the card of the etiological assessment of tamponade, which directed us to the search for multiple myeloma.
Ig A type MM appears to have more high-risk of extramedullary disease in general and pericardial involvement in particular . Cardiac tamponade secondary to myelomatous effusion of the pericardium is exceptional with only 29 cases including ours, reported in the literature and most cases are diagnosed retrospectively at postmortem examination, it is usually the result of metastatic or relapsed myeloma [3, 15–20]. As myelomatous effusion is associated with poor prognosis with an average survival of 13.5 weeks , an efficacy, less invasive procedure that can establish an accurate diagnosis with a minimum risk and discomfort is highly desirable. Positron emission tomography with 18F-fluorodeoxyglucose (18F-FDG) and computed tomography (PET-CT) are used in valid scores established in differentiating malignant pleural effusion from benign effusions [21, 22]. However, the literature on the use of PET/CT in pericardial disease remains very limited, and this area warrants further investigation [22, 23].
No current consensus exists on the management of pericardial involvement in MM. Drainage is required for initial management of symptomatic effusions and can be completed by pericardiocentesis or pleuropericardial window. Beyond pericardiocentesis, intrapericardial therapeutic administration of cytotoxic agents (bleomycin or cyclophosphamide), systemic chemotherapy (corticosteroid, vinsristin, adriamycin, cyclophosphamide, imid, bortezomib) and radiation therapy have all been used but with little therapeutic benefits, no case of hematopoietic stem cell transplant was published [17–20].
Our patient was given MPT regimen and her results after induction chemotherapy were promising (ratio FLC: 86%, no recurrence of pericardial effusion). However, her myelomatous pericardial effusion, her renal failure and hypercalcemia causing her death despite her ongoing treatment reflect the highly aggressive disease presentation at both the time of diagnosis and progression.
The present case is unique as the plasma cell-based pericardial effusion leading to cardiac tamponade is the initial presentation of MM in a patient with no signs or symptoms of myeloma.
It highlights the need of hematologists to be aware of such a possibility and investigations on pericardial fluids are necessary despite the absence of symptoms of myeloma.