An unexpected cause of the "shortness of breath" referral: a unique presentation of plasma cell dyscrasia

Andrea Ramirez, Frank Zhang, Saud Q. Alrawaf,Prathik Krishnan,Poornima Ramadas

CHEST(2023)

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摘要
SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 11 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Metastatic pulmonary calcification (MPC) typically presents in patients with medical conditions associated with hypercalcemia and rarely causes symptoms. We present a unique presentation of plasma cell dyscrasia with symptomatic MPC or pulmonary amyloidosis. CASE PRESENTATION: 59 year old male with no significant medical history who presented to his primary care physician for new dyspnea on exertion. He was previously able to mow his lawn at ease, but over the past year started to notice dyspnea. He denied ever smoking, occupational exposures and pertinent family medical history. Due to this clinical change, CXR was obtained and showed bilateral perihilar airspace opacities. As this was nonspecific, CT chest was recommended and exposed diffuse bilateral calcification, heterogeneous bone marrow and mediastinal lymphadenopathy. At this time, multiple myeloma versus other malignancy led the differential. Labs were notable for calcium 10 mg/dL, phosphorus 2.6 mg/dL, PTH 45 pg/mL, and normal kidney function. Further evaluation resulted in IgG kappa M spike of 1.2 g/dL and kappa light chains of 30 with K/L of 67, bone marrow biopsy had features consistent with plasma cell dyscrasia and a negative bone scan. PET showed intensely FDG avid bilateral pulmonary calcinosis and mediastinal lymphadenopathy. Though patient met criteria for smoldering myeloma, due to unusual presentation with associated lymphadenopathy, patient underwent EBUS. Pathology revealed plasma cell myeloma with amyloid deposition. DISCUSSION: Pulmonary calcifications are caused by two mechanisms: dystrophic form and metastatic form. Dystrophic calcifications present in damaged lung, following an inflammatory process with local and organized pattern and normal calcium levels. In contrast, MPC is typically caused by high levels of serum calcium and phosphate, which deposit in normal lung tissue. This is most commonly seen in patients with chronic kidney disease, but can also be due to hyperparathyroidism, sarcoidosis, and malignancy. There are few unique things in this case. First, patients with systemic light chain amyloidosis don't present with lung as the only or first organ to be involved, usually starts kidney or cardiac. Although we did not biopsy the lung to differentiate if this is pulmonary amyloid or calcifications, the CT showing calcifications and absence of typical amyloid lung pattern is consistent with MPC but amyloid cannot be ruled out. Second, the patient was initially classified smoldering myeloma due to presence of only 10 percent plasma cells in bone marrow without CRAB criteria, but smoldering myeloma doesn't have lymphadenopathy or plasma cells in the lymph node. You could say an unusual case of plasma cell dyscrasia. CONCLUSIONS: MPC typically has minimal pulmonary manifestations. However, this patient's presentation of dyspnea ultimately led to diagnosis of plasma cell dyscrasia. REFERENCE #1: Lakshmana Perumal N, Selvi J, Sahoo JP, et al. An unusual cause of pulmonary calcification. BMJ Case Reports CP 2019;12:e229725. REFERENCE #2: Metastatic pulmonary calcification: state-of-the-art review focused on imaging findings. Belém LC, Zanetti G, Souza AS Jr, et al. Respir Med. 2014;108:668–676 REFERENCE #3: Calcium deposition with or without bone formation in the lung. Chan ED, Morales DV, Welsh CH, McDermott MT, Schwarz MI. Am J Respir Crit Care Med. 2002;165:1654–1669. DISCLOSURES: No relevant relationships by Saud Alrawaf No relevant relationships by Prathik Krishnan No relevant relationships by POORNIMA RAMADAS No relevant relationships by Andrea Ramirez No relevant relationships by Frank Zhang
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关键词
plasma cell dyscrasia,breath,shortness
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