INTRODUCTION: Involvement of the respiratory system is unusual in Multiple Myeloma (MM). Patients with advanced MM can have lytic lesions in the bone leading to Myelomatous Pleural Effusion (MPE) and local pleural disease. There can also be mediastinal involvement with Extramedullary Plasmacytomas (EP) leading to pleural effusions. Simultaneous occurrence of both MPE and EP as the initial presentation for MM is extremely rare. We report a case with a large pleural effusion and mediastinal plasmacytoma as the initial presentation of MM.
CASE PRESENTATION: 74 year old female, past history of renal cell cancer (RCC) who had undergone a nephrectomy 3 years back and had a history of lumbar spine laminectomy and fusion secondary to degenerative disc disease 6 years back, presented to the hospital with a complaint of 3 months of worsening dyspnea on exertion and back pain. CT of the chest revealed a large right-sided effusion, pleural nodularity and large paraaortic and AP window lymph nodes along with a large paraspinal density surrounding the T4 vertebra. The effusion was tapped, found to be consistent with an exudate, and cytology showed numerous plasma cells with large multinucleated cells with open chromatin and prominent nucleoli. Immunohistochemical stains were also compatible with myeloma cells. Due to the history of RCC, the mediastinal nodes were also biopsied and the pathology found to be consistent with an extramedullary plasmacytoma. The patient underwent a bone marrow biopsy that showed variably cellular marrow with diffuse and nodular infiltrates of atypical plasma cells (30% of overall) consistent with plasma cell neoplasm. A serum protein electropheresis (SPEP) showed a monoclonal protein spike (IgG kappa, 26.1) and she was diagnosed with MM.
DISCUSSIONS: MM comprises 1% of all malignancies and 10% of all hematologic malignancy. Pleural effusions occur in about 6% of patients with MM myelomatous involvement occurs in less than 1% of cases. The majority of these patients have IgA type of monoclonal protein and the etiology of MPE is thought to be due to invasion from skeletal lesions, chest wall invasion of mediastinal masses or direct tumor deposits in the pleura. MPE is usually a late manifestation of the disease process and is associated with a more aggressive form of MM. The prognosis of these patients is very poor, with less than 4 months survival from the time of development of MPE according to published reports. EP is also a rare occurrence and only about 5% of patients with EP have concurrent MM. Most EP are found in the aerodigestive tract but mediastinal involvement is extremely rare. Our patient was unusual in that she not only presented with MPE and extramedullary plasmacytoma at the time of diagnosis of MM, but, also had IgG Kappa gammopathy that is not usually associated with pleural effusions. Pleurodesis and chemotherapy have been used for patients with MPE, however their survival is very short despite treatment. Our patient decided to choose hospice care and was discharged home where she passed away 8 weeks after diagnosis.
CONCLUSION: When faced with a pleural effusion, MPE must be considered in the differential and every effort made to accurately diagnose underlying MM. Patients with MM who do develop MPE have a worse prognosis and do not seem to respond well to currently available chemotherapeutic regimens.
DISCLOSURE: Amik Sodhi, No Financial Disclosure Information; No Product/Research Disclosure Information