RESUMO
Background: Waldenstrom's disease is characterized by the presence of pathological changes in the B lymphocytes that are in the last stages of maturation. One characteristic of WM is the production of an abnormal high amount of IgM and hyper viscosity syndrome. The MW gets worse, symptoms such as fatigue, weight loss, night sweats, fever, recurrent infections and swollen lymph nodes develop in patients who have a known history of MGUS. In this clinical case, our patient without history of MGUS, presents for the first time for medical observation only for ascites and the presence of an interportocaval lymph node package. An atypical presentation of the disease that makes us reflect on the difficulty of making a diagnosis in the elderly patient and on pathogenetic hypotheses of ascites not yet explored. Case Presentation: Seventy-three-year-old patient, hospitalized for the onset of ascites with sloping edema, diffuse left pulmonary opacification. At the ultrasound check, cava and portal vessels patent and of regular caliber, however with inversion of flow in correspondence with the right branch and of the door to the hilum, with a subdiaphragmatic retrocaval focus with a maximum diameter of about 3 cm, which cannot be better viewed. CT scan of the abdomen with confirmation of the presence of an interportocaval lymph node package. After evidence of the electrophoretic protein picture of a double component, probably monoclonal with positive urinary immunofixation for free K chains. IgM dosage equal to 2190 mg. Serum immunofixation practice that confirms the diagnosis of type B lymphoproliferative syndrome as per Waldenstrom's disease, confirmed by bone marrow aspiration with morphological and flow cytometric study. Immediately begin chemotherapy with Bendamustine 120 mg. After 4 weeks of therapy with the reduction of IgM values, the patient no longer presented ascites. Conclusion: This case has an unusual presentation of this disease and we could shed a new light on the possible pathogenesis of portal hypertension in Waldenstrom'disease.
RESUMO
PURPOSE: The Italian Tailored Assessment of Lung Indeterminate Accidental Nodule (ITALIAN) trial is a trial drawn to determine the performance of 18F-FDG-PET/CT in patients with solitary pulmonary nodules (SPN), stratified for a different kind of risk. An additional end-point was to compare the diagnostic information and estimated dosimetry, provided by a segmental PET/CT (s-PET/CT) acquisition instead of a whole body PET/CT (wb-PET/CT), in order to evaluate if segmental thoracic PET/CT can be used in patients with SPN. METHODS: 18F-FDG PET/CT of 502 patients, stratified for pre-test cancer risk, was retrospectively analyzed. FDG uptake in SPN was assessed by a 4-point scoring (4PS) system and a semiquantitative analysis using the ratio between SUVmax in SPN and SUVmean in mediastinal blood pool (BP), and between SUVmax in SPN and SUVmean in the liver (L). Histopathology and/or follow-up data were used as a standard of reference. Data obtained on the thoracic part of wb-PET/CT, defined as s - PET/CT, were compared with those deriving from wb-PET/CT. RESULTS: SPNs were malignant in 180 patients (36%), benign in 175 (35%), and indeterminate in 147 (29%). The 355 patients diagnosed with a definitive SPN nature (malignant or benign) were considered for the analysis of PET performance. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values, and accuracy were 85.6%, 85.7%, 86%, 85.2%, and 85.6%, respectively. Sensitivity and PPV were higher in intermediate and high-risk patients. 18F-FDG uptake indicative of thoracic and extra-thoracic lesions was detectable in 13% and 3% of the patients. Compared to wb-PET/CT, s-PET/CT could save about 2/3 of 18F-FDG dose, radiation exposure or scan-time, without affecting the clinical impact of PET/CT. CONCLUSION: In patients with SPN, the pre-test likelihood of malignancy stratification allows to better define PET clinical setting and its diagnostic power. In subjects with low-intermediate pre-test likelihood of malignancy, s-PET/CT might be planned in advance. The adoption of this segmental strategy could reduce radiation exposure, scan-time, and might allow individually targeted protocols.