Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

Indranee Rajapreyar; Joanna Joly; Jose Tallaj; Salpy V Pamboukian; Ayman Haj Assad; Carrie Lenneman; Silvio Litovsky; Arka Chatterjee; Charles Hoopes; Andrew Lenneman

doi:10.1016/j.mayocpiqo.2020.09.004

Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

Mayo Clin Proc Innov Qual Outcomes. 2020 Nov 20;5(1):210-218. doi: 10.1016/j.mayocpiqo.2020.09.004. eCollection 2021 Feb.

Affiliations

¹ Division of Cardiovascular Disease, Section of Advanced Heart Failure and Transplantation, University of Alabama at Birmingham, Birmingham.
² Division of Cardiovascular disease, University of Alabama at Birmingham, Birmingham.
³ Department of Pathology, University of Alabama at Birmingham, Birmingham.
⁴ Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham.

Abstract

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.

Keywords: AL, amyloid light chain; ASCT, autologous stem cell transplant; BMB, bone marrow biopsy; CKD, chronic kidney disease; CT, computed tomography; FLC, free light chain; HIV, human immunodeficiency virus; ILD, interstitial lung disease; LC-MGUS, light chain monoclonal gammopathy of undetermined significance; LCDD, light chain deposition disease; LV, left ventricular; MGUS, monoclonal gammopathy of undetermined significance; MM, multiple myeloma; MRI, magnetic resonance imaging; PAP, pulmonary artery pressure; PH, pulmonary hypertension; RA, right atrial; RHC, right heart catheterization; RV, right ventricle/ventricular; TTE, transthoracic echocardiography; WHO, World Health Organization.

Publication types

Case Reports