Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

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.

Figure 1

A, A 2-dimensional echocardiogram revealing right ventricular dilation and pericardial effusion. B, A year later, a 2-dimensional echocardiogram revealing severe left ventricular hypertrophy suggestive of infiltrative disease.

Figure 2

Sections reveal a hypercellular marrow containing a large number of plasma cells (arrows).

Figure 3

A, Congo red–stained section reveals amorphous material consistent with amyloid in the myocardial interstitium (arrows). B, Under polarized light, apple green birefringence is seen (arrows).