On Pulmonary Amyloidosis as the Presenting Feature of Multiple Myeloma

Document Type

Conference Proceeding

Publication Date

5-17-2022

Publication Title

Am J Respir Crit Care Med

Abstract

Introduction Primary systemic amyloidosis is a rare multi-systemic disease with a prevalence ranging between 6.1 and 10.5 per million person-years. In amyloidosis, the precursor amyloidogenic protein accumulates, either locally or systemically, resulting in end-organ-damage. Pulmonary involvement in amyloidosis is reported in approximately 50% of the patients, but rarely symptomatic. Manifestations include tracheobronchial infiltration, pleural effusions, pulmonary vascular disease, nodular pulmonary amyloidosis, and diffuse alveolar septal amyloidosis (DASAL). Herein, we report a case of pulmonary amyloidosis (P-AL) as the presenting feature of Multiple Myeloma (MM), which is seldom reported in literature. Case Presentation 62 year old male with a past history of hypertension and obstructive sleep apnea presented with progressively worsening exertional dyspnea (MMRC-3) and dry cough for 1 year. Review of systems, social/occupational/environmental exposure history, and physical examination were noncontributory. Cardiac workup, including echocardiogram, and autoimmune serology were unremarkable. High Resolution Computed Tomography (HRCT) showed diffuse upper lobe and peripheral-prominent ground glass opacities (GGO), subtle reticulation, sub-pleural sparing, without air trapping or honeycombing. Pulmonary function testing (PFT) revealed severe diffusion impairment and an FVC of 3.71 L. Surgical lung biopsy showed evidence of Nonspecific Interstitial Pneumonia and Congo-Red staining was positive for apple-green birefringence, consistent with DAS-AL with predominant peri-vascular deposition. Per ILD Mutli-Disciplinary-Meeting recommendations, a hematological workup was performed that revealed markedly elevated lambda light chains (163 mg/dL). Subsequent bone marrow biopsy (BMBx) and flow-cytometry showed lamda light chain restricted plasma cells contributing 15-20% of bone marrow cellularity, confirming the diagnosis of MM. Patient received two cycles of CyBor-D regimen (cyclophosphamide, bortezomib, and dexamethasone) followed by VRd regimen (lenalidomide and dexamethasone). Repeat BMBx revealed decreased MM burden, following which the patient underwent autologous stem cell transplant (ASCT) and is currently under surveillance. On pulmonary follow-up visits, the patient reported significant symptomatic improvement (MMRC 0 - 1). Repeat HRCT showed interval improvement of GGOs and repeat PFT showed improvement in diffusion and FVC of 5.31 L. Discussion Our case sheds light on the observation that treatment of MM with chemotherapy and ASCT led to marked reduction in MM burden with a corresponding improvement in P-AL corroborated by symptomatic, radiological, and physiological response. Post-treatment pulmonary evaluation to assure clinical stability is essential and pathologic confirmation of amyloidosis resolution may be warranted in specific clinical scenarios. Physicians should be cognizant of the importance of identifying the underlying etiology of pulmonary amyloidosis since treatment of underlying etiology can lead to resolution of amyloidosis.

Volume

205

Issue

1

First Page

A2765

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