A 64-year-old Caucasian male presented to the emergency room complaining of progressive fatigue, light-headedness, shortness of breath, and an intermittent cough. He reported waking up with extreme malaise and inability to ambulate on the day of presentation. However, upon further questioning, it became evident that his symptoms had started 3 months prior and had gradually progressed in severity. He had been on a camping trip in the Rocky Mountains of Colorado and stayed in a yurt for one and half months before presentation; the trip was cut short due to his shortness of breath and decreased exercise tolerance. He had seen his primary care provider for these symptoms and a chest X-ray showed bilateral upper and lower lobe alveolar infiltrates. He was treated for atypical pneumonia with a 5-day course of azithromycin. His cough resolved but there was no improvement in the rest of his symptoms. Review of systems was significant for unintentional weight loss of 8–10 pounds over a period of 2 years; he denied nausea, vomiting, diarrhea, and/or melena.\nHis medical history included hypertension, glaucoma, and hyperlipidemia. He was a former smoker, having quit about 20 years prior. He drank alcohol occasionally. Family history was significant for leukemia in his father.\nPhysical examination was significant for pale skin and conjunctiva and a 3/6 systolic murmur best heard at the left sternal border.\nLaboratory investigations showed hematocrit of 11.3% and hemoglobin of 3.7 gm/dl, and the stool guaiac test was positive. Computed tomography (CT) of the chest with intravenous contrast showed bilateral pulmonary opacities and mediastinal lymphadenopathy in the perivascular and posterior mediastinum (). CT of the abdomen and pelvis with contrast showed severe gastric wall thickening involving the proximal body and fundus with surrounding inflammatory changes (). Esophagogastroduodenoscopy with biopsy was performed. Gastric pathology showed an intense infiltrate of small, mature lymphocytes extending to the epithelium and obliteration of normal glandular architecture; immune-histochemical staining showed CD20+ and abnormal dim CD43 co-expression ( and ) and BCL2+ and low to moderate Ki-67 proliferation index suggestive of extra-nodal marginal zone B-cell lymphoma of mucosal-associated lymphoid tissue. Helicobacter pylori was not identified on immunohistochemical staining and a serological analysis of IgG antibodies was negative ruling out previous exposure. A HLA-DQ pattern analysis for celiac disease was inconclusive. Serological analysis to rule out chronic infective etiology for immune stimulation from blastomyces, coccidioides, histoplasma, legionella, mycoplasma, aspergillus, and hepatitis B or C was negative. Subsequently, he also underwent bronchoscopy with transbronchial biopsy of the right upper and middle lobes, which showed atypical lymphoid infiltrate consistent with involvement from known MALT lymphoma with CD20+ and dim CD43 co-expression ( and ). He was treated symptomatically for anemia with blood transfusion and intravenous iron administration. He underwent radiation therapy for MALT lymphoma of the stomach due to gastric bleeding and severe anemia requiring multiple transfusions. Radiation treatment was given with 15 MV photons with an AP/PA treatment technique using MLC shaping with CT based three-dimensional treatment and planning. He received a total dose of 36.0Gy in 20 fractions over 27 elapsed days. Immunotherapy with rituximab was considered. However, since the only symptomatic manifestations were related to his severe anemia secondary to the gastric involvement of his lymphoma, he was treated as localized disease. He is been closely followed by his oncologist and has been undergoing surveillance CT-scan of his chest/abdomen every 6 months and yearly PET scan. He has not required any chemotherapy or further radiation therapy and his lymphoma has remained stable.
A 59-year-old, former smoker, man, presented at the Emergency Department with a sub-acute onset of increasing dyspnea and persistent dry cough. Previous medical history consisted of an occupational exposure to fine dusts and other toxic inhalants (ex pottery maker), and acute myocardial infarction previously treated with PTCA. No symptoms of collagen vascular/autoimmune disease were present.\nAt physical examination, digital clubbing was observed and inspiratory basal crackles were detected. Chest X-rays documented multiple bilateral pulmonary consolidations and a High-Resolution Computed Tomography (HRCT) confirmed the presence of bilateral multiple areas of consolidations with frequent air bronchograms, interlobular septal thickenings and ground-glass opacities (). Imaging was consistent with a crazy paving pattern.\nSputum examination was negative for pathogenic microbes, while pulmonary function tests revealed a moderate restrictive disease (VEMS 66,5%, FVC 61,1%, TLC 56,8%).\nThe post-operative course was unremarkable and the patient was discharged in 4th post-op day. At histology, the lung parenchyma showed nodular lesions characterized by an infiltration monomorphic small lymphocites and plasma-cells obliterating the alveolar spaces () associated with lymphoepithelial lesions (B). At immunohistochemistry, neoplastic lymphocytes show a B-cell clonal phenotype (CD20 immunohistochemistry, Hematoxylin counterstain, 20X (C)); moreover, stain for MIB1 shows a low proliferative index of the neoplastic infiltrate (MIB1 immunohistochemistry, Hematoxylin counterstain, 20X (D)) accounting for 3-5%. The final diagnosis of mucosa-associated lymphoid tissue-derived (MALT) lymphoma was finally made. The patient underwent chemotherapy with Rituximab and Bendamustine, with gradual and progressive improvement of the overall clinical scenario.