Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

• Main Authors: Indranee Rajapreyar, MD, Joanna Joly, MD, Jose Tallaj, MD, Salpy V. Pamboukian, MD, Ayman Haj Assad, MD, Carrie Lenneman, MD, Silvio Litovsky, MD, Arka Chatterjee, MD, Charles Hoopes, MD, Andrew Lenneman, MD
• Format: Article
• Language: English
• Published: Elsevier 2021-02-01
• Series: Mayo Clinic Proceedings: Innovations, Quality & Outcomes
• Online Access: http://www.sciencedirect.com/science/article/pii/S2542454820301879
• ISSN: 2542-4548

Pulmonary hypertension (PH) has been described in myeloproliferative disorders; monoclonal plasma cell disorder such as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome; and plasma cell dyscrasias such as multiple myeloma and amyloidosis. We describe 4 cases of PH likely due to pulmonary vascular involvement and myocardial deposition from light chain deposition disease, amyloidosis, and multiple myeloma. On the basis of our clinical experience and literature review, we propose screening for plasma cell dyscrasia in patients with heart failure with preserved ejection fraction, unexplained PH, and hematological abnormalities. We also recommend inclusion of cardiopulmonary screening in patients with monoclonal gammopathy of undetermined significance.