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Fibrosing pleuritis is a condition not widely reported or well understood, and is thought to develop in response to recurrent pleural inflammation associated with exudative effusions. In Waldenström's macroglobulinemia (WM), patients with extramedullary pulmonary involvement are sparingly mentioned as a cause of fibrosing pleuritis in literature. We discuss a rare presentation of fibrosing pleuritis in a patient with Waldenström's macroglobulinemia-associated pleural effusions.CASE PRESENTATION: An 88 year old male presented with shortness of breath with a CTA chest showing a moderate rightsided pleural effusion, para-aortic lymphadenopathy and T-5 vertebral body lucencies. A diagnostic thoracentesis revealed an exudative fluid with elevated protein of 5.1 g/dL and negative cytology. Serum protein electrophoresis showed an M spike of 1.2 g/ dL, immunofixation showed monoclonal IgM lambda, quantitative IgM was elevated at 2079 mg/dL and bone marrow biopsy revealed metastatic prostate adenocarcinoma and lymphoplasmacytic lymphoma (LPL). A month later, he presented with similar complaints and findings of recurrent effusions necessitating a right-sided VATS, decortication and pleurodesis. The pleural biopsy was reported as fibrosing pleuritis, with genetic pleural fluid analysis showing positivity for MYD88 gene mutation, suggestive of Waldenström's macroglobulinemia.DISCUSSION: Waldenström's macroglobulinemia is a rare cancer characterized by an infiltration of the bone marrow by IgMsecreting lymphoplasmacytic cells, and it is the most common type of LPL. Apart from bone marrow involvement, there are a few reports of pulmonary involvement with presentations ranging from lung nodules, diffuse infiltrates to pleural effusions. Attempting to establish a malignant etiology for pleural effusion and fibrosing pleuritis in WM can be challenging, and standard techniques may be insensitive. Diagnosis is made by identifying IgM monoclonal protein and greater than 10 % of clonal lymphoplasmacytic cells in the bone marrow, with MYD88 L265P mutation found to be positive in 93-97% of patients. PCR detection of this mutation had a 100% sensitivity and 92.1% specificity for WM based on one study. Treatment decisions of WM are based on symptoms, with prospective studies demonstrating durable response to Ibrutinib monotherapy; the greatest effect seen in patients with MYD88 mutation. Our patient showed MYD88 mutation positivity and was treated successfully with Ibrutinib.CONCLUSIONS: Malignant pleural effusions and resultant fibrosing pleuritis are a rare extramedullary pulmonary manifestation of Waldenström's macroglobulinemia.Greater than 90 % of these patients have MYD88 gene mutations, with growing evidence that testing for pleural fluid can point towards the diagnosis of a malignant pleural effusion in the absence of positive cytology findings of WM, and aid prompt treatment.

INTRODUCTION: Scimitar syndrome (SS) is a rare congenital disease. It is characterized by abnormal drainage of right pulmonary vein into inferior vena cava or right atrium instead of left atrium. It is commonly associated with hypoplastic right lung and right pulmonary artery as well as dextroposition of the heart. The name of the syndrome is derived from the radiographic manifestation of the anomalous vein resembling a Turkish sword. CASE PRESENTATION:A 45 years old woman with history of obstructive sleep apnea, hypertension, morbid obesity, recurrent mild respiratory infections since childhood. She complained of cough and dyspnea on exertion for four months. Over that period, she was repeatedly treated with several courses of antibiotics and steroids. During this period, she also gained 40 pounds. With progression of shortness of breath and need for supplemental oxygen, she was referred to pulmonology clinic. Chest CT with contrast reported hypoplastic right lung with a prominent right pulmonary vein draining into the junction of inferior vena cava and right atrium, compatible with Scimitar Syndrome. On further workup, PFT showed moderate restrictive pattern, echocardiogram showed evidence of mild pulmonary hypertension. She was then referred to a tertiary center and eventually underwent surgical anastomosis of the Scimitar vein via the present atrial septal defect into the left atrium, with patch enlargement of the ostium of the scimitar vein at its entrance, and tricuspid valve repair commissuroplasty. After surgery, her dyspnea improved significantly from having dyspnea at rest to having dyspnea only with moderate activity. supplemental oxygen was weaned to continuous 2 liters of nasal canula. She continues to improve with cardiopulmonary rehabilitation and has recently returned to work. DISCUSSION: SS accounts for 0.5-1 percent of all congenital heart disease cases. The prevalence of SS is estimated to be 1-3 of every 100,00 livebirths. Two types of SS have been described, the infantile and adult forms. Infantile form is usually associated with other thoracic and vascular abnormalities. Adult form has a smaller shunt and is more commonly isolated. Atrial septal defect is the most common associated cardiac anomaly in both adult and infantile forms. Echocardiogram with color doppler studies as well as computed tomography are the mainstay diagnostic tools. Most patients are asymptomatic at birth. Clinical symptoms are similar to ASD. Exercise intolerance is the most common complaint. Depending on the shunt volume, patients may range from completely asymptomatic to having dyspnea and recurrent respiratory infections.CONCLUSIONS: Scimitar syndrome is rarely seen in adulthood and can present as a progressive disease. Many types of surgical repair have been proposed. Most repairs are designed to divert the anomalous connection between pulmonary veins and systemic veins and drain the flow to left atrium.