A Case of Recurrent Localized Pulmonary Nodular Light Chain Amyloidosis Treated with Daratumumab plus CyBorD.

INTRODUCTION: Nodular pulmonary amyloidosis (NPA) is a localized form of light chain (AL) amyloidosis often found incidentally and typically has an indolent and benign disease course treated with resection or local excision. We present a patient with recurrent localized AL amyloidosis who required further treatment. CASE PRESENTATION: A 63-year-old female with monoclonal gammopathy of undetermined significance (MGUS) was found to have pulmonary AL amyloid on wedge resection and later had recurrence. The patient did not have signs of clonal plasma cell proliferation or systemic AL amyloid. She was treated with daratumumab, cyclophosphamide, bortezomib, and dexamethasone. After initiation of treatment, the patient has had significant hematologic and radiographic response. CONCLUSION: The patient had NPA recurrence with organ dysfunction without systemic disease. Because the presentation of recurrent pulmonary AL amyloidosis is rare, there is no published evidence on treatment. However, the patient has had hematologic and radiographic improvement after initiating treatment with a systemic protocol.

Quality of transbronchial biopsy with large forceps compared to cryobiopsy: a randomized controlled, single blinded live animal study.

Background: Transbronchial forceps biopsy is the widely accepted modality for obtaining tissue specimens for the evaluation of unexplained lung parenchymal abnormalities. However, cryoprobe biopsy provides large specimen sizes and higher yield performance. Utilization of cryoprobe biopsy remains limited by its need to be performed under rigid bronchoscopy and subsequent required operator expertise. We evaluated whether a larger, 2.8 mm forceps could be utilized for parenchymal biopsies. A larger size would surrogate the cryoprobe's large sample size and forceps mechanism to obviate the need for rigid bronchoscopy and its requirement for removing the sample en bloc. Methods: This prospective, randomized controlled, single-blinded porcine study compared a 1.9 mm cryoprobe, a 2.4 mm cryoprobe, and a 2.8 mm forceps. Assessment of histopathologic quality, sample quality and surface area, attempts to retrieve specimen samples, fluoroscopy activation time, overall procedural time, and complications were compared. Results: Although cryoprobe yielded larger specimens, there was no statistical difference amongst all tools with respect to alveolar tissue surface area. There was bleeding on all cryoprobe biopsies. No bleeding was observed with forceps. Out of 32 potential combinations of interventions for bleeding control, 18 (56.3%) were made. There was no significant difference in sample quality between all three modalities. There was one pneumothorax in the forceps arm. Conclusions: Large forceps (LF) biopsy is a feasible technique while providing high diagnostic yield without the need for advanced therapeutic tools. Human studies are needed to further corroborate this technique.

Cryotherapy: A Safe Approach to Pulmonary Hemorrhage During VV-ECMO.

The number of hospitals with veno-venous extracorporeal membrane oxygenation (VV-ECMO) capabilities is expanding. To support an ECMO program, centers must be equipped to handle associated complications such as pulmonary hemorrhage. We describe a case series of 4 patients with life-threatening pulmonary bleeding and central airway obstruction. A therapeutic approach of anticoagulation cessation coupled with cryoextraction via flexible bronchoscopy led to successful restoration of airway patency without any adverse events. A low threshold to stop anticoagulation with a strong consideration of bronchoscopy with cryotherapy for pulmonary toilet should be done in patients with pulmonary hemorrhage during VV-ECMO.

Therapeutic closure of bronchopleural fistulas using ethanol.

Bronchopleural fistula (BPF) leading to persistent air leak (PAL), be it a complication of pulmonary resection, radiation, or direct tumor mass effect, is associated with high morbidity, impaired quality of life, and an increased risk of death. Incidence of BPF following pneumonectomy ranges between 4.4% and 20% with mortality ranging from 27.2% to 71%. Following lobectomy, incidence ranges from 0.5% to 1.5% in reported series. BPFs are more likely to occur following right-sided pneumonectomy, while patients undergoing bi-lobectomy were more likely to suffer BPF than those undergoing single lobectomy. In addition to supportive care, including appropriate antibiotics and nutrition, management of BPF includes pleural decontamination, BPF closure, and ultimately obliteration of the pleural space. There are surgical and bronchoscopic approaches for the management of BPF. Surgical interventions are best suited for large BPFs, and those occurring in the early postoperative period. Bronchoscopic techniques may be used for smaller BPFs, or when an individual patient is no longer a surgical candidate. Published reports have described the use of polyethylene glycol, fibrin glues, autologous blood products, gel foam, silver nitrate, and stenting among other techniques. The Amplatzer device, used to close atrial septal defects has shown promise as a bronchoscopic therapy. Following their approval under the humanitarian device exemption program for treatment of prolonged air leaks, endobronchial valves have been used for BPF. No bronchoscopic technique is universally applicable, and treatment should be individualized. In this report, we describe two separate cases where we use an Olympus© 21-gauge EBUS-TBNA (endobronchial ultrasound-transbronchial needle aspiration) needle for directed submucosal injection of ethanol leading to closure of the BPF and subsequent successful resolution of PAL.

A 49-Year-Old Man With Cough and Hand, Wrist, and Knee Pain.

CASE PRESENTATION: A 49-year-old man was sent by his primary care physician to the rheumatology clinic with complaints of several months of bilateral lower extremity swelling. The swelling migrated from both ankles up to his knees. Presenting symptoms consisted of bilateral knee pain as well as bilateral wrist and hand pain with swelling. Pulmonary symptoms consisted of a nagging productive cough of several months. He also complained of significant weight loss: 50 pounds over 12 months. He was a never smoker. The examination was notable for bilateral knee effusions. Radiographs of his wrists, hands, and knee were obtained, along with a chest radiograph. He was then referred to a pulmonologist for further workup.