Conference: 2014 International PHA Conference and Scientific Sessions
Release Date: 06.21.2014
Presentation Type: Abstracts
File Download: 2014 Conference Abstract - Eric Fendstad II
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Patients with chronic myeloproliferative disorders may develop pulmonary hypertension (PH). While PH has been described in association with multiple myeloma, little is known about reversible PH in this setting.
Background: Patients with chronic myeloproliferative disorders may develop pulmonary hypertension (PH). While PH has been described in association with multiple myeloma, little is known about reversible PH in this setting. We report two cases of reversible PH in the setting of smoldering multiple myeloma (SMM) that resolved with chemotherapy.
Clinical Presentation: Patient #1 is 63 year old male who presented with dyspnea and increased abdominal girth. Serum protein electrophoresis with immunofixation demonstrated an M-spike of 2.1. An elevated lambda free light chain of 53.6 mg/dl (normal 0.57-2.63) while urine protein electrophoresis had an M-spike of 35 mg consistent with SMM. Echocardiography demonstrated a right ventricular systolic pressure (RVSP) of 84 mmHg and right atrial pressure of 20 mmHg. Right heart catheterization confirmed PH with a mean pulmonary artery pressure of 40 mmHg, pulmonary capillary wedge pressure of 10 mmHg, and a pulmonary vascular resistance (PVR) of 4.72 Wood Units. The patient received Bortezomib and Ambrisentan with resolution of SMM and dyspnea as the RVSP decreased to 43 mmHg. He underwent successful autologous stem cell transplant with remission and remains dyspnea-free with RVSP < 40 mmHg follow up echocardiograms for the last three years on Ambrisentan.
Patient #2 is a 59 year old male who presented with upper and lower extremity neuropathy, dysphagia, skin thickening, and exertional dyspnea with one flight of stairs. Evaluation included a serum protein electrophoresis demonstrating an M-spike of 2.3. Bone marrow biopsy demonstrated 12% plasma cells. Workup for amyloidosis was negative. Echocardiogram demonstrated moderate right ventricular enlargement with decreased systolic function, an RVSP of 106 mmHg, and right atrial pressure of 14 mmHg. Right heart catheterization demonstrated a mean pulmonary artery pressure of 52 mmHg, pulmonary capillary wedge pressure of 6 mmHg, and PVR of 9.93 Wood Units. He was diagnosed with SMM and secondary scleromyxedema, started on intravenous epoprostenol and remained on it for one year. Despite autologous stem cell transplant, his symptoms remained and he was started on cyclic Dexamethasone for one year, Thalidomide for two years, and Sildenafil for five years. Four years after the stem cell transplant, his M-spike was undetectable and RVSP had normalized to 30 mmHg, all of which correlated with symptom resolution. He remains free of any PH symptoms at eight years post stem cell transplant follow up with normal RVSP on only Diltiazem.
Conclusions: Development of SMM may predispose to PH and should prompt screening echocardiography. Pulmonary Hypertension in the setting of SMM also represents a potentially reversible cause when treated.
Type: Case Study