Pericardial relapse of multiple myeloma

Abstract

In patients who experience relapse of multiple myeloma, upwards of 30% can have extramedullary disease. The presence of extramedullary multiple myeloma is typically associated with adverse cytogenetics and a poor prognosis. Organs most commonly involved include the liver, skin, central nervous system, pleural effusions, kidney, lymph nodes, and pancreas. We present the case of a 53-year-old man with IgA kappa multiple myeloma with the adverse cytogenetic findings of t(4;14) and 1q21 gain who had achieved a stringent complete (sCR) response after initial therapy with carfilzomib, lenalidomide and dexamethasone. Stringent complete response is defined as the normalization of the free light chain ratio in the serum and an absence of clonal cells in the bone marrow in additiion to criteria needed to achieve complete response. Prior to undergoing a planned autologous stem cell transplant, this patient experienced cardiac tamponade secondary to extramedullary relapse of his multiple myeloma which was limited to the pericardium. In response, his treatment regimen was transitioned to pomalidomide, bortezomib, dexamethasone and cyclophosphamide for three cycles after which he again achieved sCR and ultimately underwent autologous stem cell transplant. Post-transplant consolidation therapy was administered in the form of pomalidomide, bortezomib and dexamethasone, followed by pomalidomide and bortezomib maintenance, which he has continued to receive for 3 years without evidence of disease progression.

Keywords: cancer intervention; haematology (drugs and medicines); malignant and benign haematology; malignant disease and immunosuppression.

Figure 1

Positron emission tomography/computed tomography (PET/CT) at the time of diagnosis demonstrating increased metabolic activity in the left humerus, right sacrum and right femur, and too numerous to count subcentimetre lytic skeletal lesions.

Figure 2

PET/CT at the time of isolated pericardial relapse without discrete extramedullary hypermetabolic lesions that would suggest concurrent malignancy. MIP, maximum intensity projection; WHOL, whole body scan.