LIGHT CHAIN DEPOSITION DISEASE: WHAT?

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pulmonary light chain deposition disease (PLDD) is an exceptionally rare diagnosis characterized by the of nonamyloid chains leading to progressive dyspnea and airflow obstruction. Cystic PLDD demonstrates extensive, bilateral thin-walled cysts. Pathologically, amorphous eosinophilic material surrounded giant cell reaction that does not react Congo-Red staining noted. transplantation can be curative. CASE PRESENTATION: A 38-year-old female with past medical history mixed connective tissue lupus-like features, long-term tobacco use was seen in pulmonology office for worsening on exertion dry cough. CT-Chest showed multiple function tests were unremarkable. VEGF-D, Alpha-1-antitrypsin genotype, HIV left upper lobe video assisted thorascopic lung biopsy performed. Pathology consistent pulmonary associated patchy lymphocytic infiltrates, without evidence lymphoproliferative disease. There amyloid-like nodules did Congo-Red, Langerhans Histiocytosis negative.Further work-up revealed normal counts, kidney function, a SPEP UPEP. Free Kappa/Lambda ratio slightly elevated. Bone marrow biopsy, FISH analysis, karyotype performed rule-out clonal process. These studies She referred transplant center. DISCUSSION: Light result monoclonal immunoglobulin deposits, typically involves renal, hepatic, cardiac systems. Isolated involvement rare, only recently noted phenomenon. Cystic-predominant bilaterally progressively expanding cysts are similar appearance lymphangioleiomyomatosis (LAM). Clinically, correlates obstructive PFT's. often elevated other lab abnormalities. Surgical should obtained, particularly if patient has history, as differentiating from crucial. amyloidosis, cystic walls stains positive hematoxylin eosin. The usually reactions, also airways blood vessels. However, there no Conge-Red staining. Treatment limited transplantation. CONCLUSIONS: requires surgical rheumatologic or hematologic comorbidities present. REFERENCE #1: Colombat M, Caudroy S, Lagonotte E, et al. Pathomechanisms cyst formation Eur Respir J 2008;32:1399-403. 10.1183/09031936.00132007 #2: Khawaja Shedding Disease. Expert Clinician. 2019. DISCLOSURES: No relevant relationships Kaitlyn Musco, source=Web Response Dylan Soller, Richard Strobel,