A 50-year-old man of Indian descent with a past medical history only significant for multiple myeloma was admitted to our hospital for complaints of fatigue, increasing dyspnea, and bilateral lower extremity edema, which had been progressive for the past 2 months. The multiple myeloma had been refractory to treatment with considerable progression since the diagnosis was made 3 years earlier. Over the years, he had been treated with pulse steroids and chemotherapy (CDEP: cyclophosphamide, dexamethasone, etoposide, cisplatin), followed by a peripheral stem cell transplant and then maintenance steroids. His disease continued to progress with refractory cytpopenias and extensive bone involvement. Within the year prior to this admission, he was also treated with thalidomide and, more recently, bortezomib. Despite the multitude of therapies he underwent, his myeloma remained unresponsive. On exam, he appeared to be in congestive heart failure with findings of tachycardia. Rales were heard halfway up the lung fields bilaterally, and he had a jugular venous pulse at 12 cm, ascites, and 3+ bilateral lower extremity edema, as well as scrotal edema. Laboratory values demonstrated bicytopenia with a hematocrit concentration of 27 mg/dL and a platelet count of 10 x 109 cells/L. His chemistry panel was remarkable for a BUN value and creatinine level of 55 mg/dL and 1.0 mg/dL, respectively, and a calcium level of 12.2 mg/dL. It was notable that his albumin concentration was not significantly depleted at 3.6 mg/dL. The ECG obtained in the ED demonstrated sinus rhythm with a rate of 93 bpm, normal voltage, and a left atrial abnormality. His chest radiograph was consistent with congestion. He was directly admitted to workup a new diagnosis of congestive heart failure. A two-dimensional echocardiogram with Doppler showed a left atrium that was mildly dilated, normal left ventricular wall thickness, preserved systolic function with an ejection fraction of 65%, normal diastolic function, and a right ventricular systolic pressure of 42 mm Hg. Despite aggressive diuresis, including a furosemide drip at 20 mg/h, his status continued to decline, and he went on to require mechanical ventilation support due to respiratory failure. Below is an MRI taken of the abdomen and pelvis 1 month prior to his hospitalization.
What is his diagnosis and what is the pathophysiology?
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