Background
Our case of a patient with untreated lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia with extramedullary pleural effusion is the first documented case of pleural fluid MYD88 L265P mutation status in a community hospital setting. Our patient was intolerant to 420 mg ibrutinib, but still achieved a lasting complete remission, as defined by National Comprehensive Cancer Network guidelines, with a dose reduction to 240 mg of ibrutinib.
Case presentation
A 72-year-old Caucasian (white) man diagnosed with monoclonal immunoglobin M kappa lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia monitored without treatment for 2 years, presented with dyspnea and a left pleural effusion. At presentation, computed tomography scans of his chest, abdomen, and pelvis showed layering left pleural effusion and para-aortic lymphadenopathy. Pleural fluid cytology demonstrated B-cell lymphoma of the lymphoplasmacytic subtype, with monoclonal kappa B-cell population on flow and a positive MYD88 L265P mutation. The pleural effusion recurred post-thoracentesis and he achieved a lasting complete remission as defined by National Comprehensive Cancer Network guideline with 240 mg ibrutinib.
Conclusions
Our discussion details a comprehensive literature review of extramedullary pulmonary involvement in Waldenstrom’s macroglobulinemia. Establishing a malignant etiology for pleural effusion in Waldenstrom’s macroglobulinemia can be challenging, as standard techniques may be insensitive. Allele-specific polymerase chain reaction for detecting MYD88 L265P mutations is more sensitive for confirming lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia in pleural fluid. Extramedullary pulmonary involvement usually presents post-diagnosis of Waldenstrom’s macroglobulinemia and responds well to Waldenstrom’s macroglobulinemia-directed treatment regimens. Allele-specific polymerase chain reaction is a sensitive assay for detecting MYD88 L265P mutations in pleural fluid to support the diagnosis of malignant pleural effusion in the setting of Waldenstrom’s macroglobulinemia and helps guide the treatment decision to use ibrutinib. Although intolerant of ibrutinib 420 mg, our patient achieved complete and sustained remission of pleural effusion with a dose of 240 mg with progression free survival of over 30 months.
Background: Initial evaluation and follow up of Bladder carcinoma (BC) is done by CT urograpghy or by MRI in suitable patients with BC. However, the role of FDG PET/CT still remains unclear. The aim of the present study was to evaluate the accuracy, the effect upon treatment decision, as compared to CT in patients with suspected recurrent BC. Methods: 107 patients affected by BC underwent FDG PET/CT for restaging purpose. The diagnostic accuracy of visually interpreted FDG PET/CT was assessed compared to histology (n ¼ 14), contrast-enhanced CT (n ¼ 62) and clinical follow-up (n ¼ 62). Rest of the patients who lost to follow up (n ¼ 45) were excluded from the study. A separate Post diuretic view was taken in all patients 40min after injecting 40mg of Frusemide. Sensitivity, specificity, NPV and PPV were calculated and semi quantitative analysis was done using PET values (SUVmax). Comparison with contrast enhanced CT was done. Results: PET/CT was considered positive in 39 of 62 patients. Of these, recurrent BC was confirmed in 41 (95.12%). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of PET/CT were 90%, 86%, 92%, 83%, 89%. As compared to pre lasix view, post lasix view median SUVmax was higher in patients and aided in identifying local recurrences better (n ¼ 5, p < 0.05). As compared to contrast enhanced CT, PET/CT fared well in correctly identifying local recurrence (p < 0.01) and lymph nodal metastases (p < 0.01).PET/CT findings altered the management in 22 patients (modified therapy in 14 PET-positive patients, watch-and-wait in 8 PET-negative patients. Conclusions: As compared to Contrast enhanced CT, FDG PETCT (post Lasix) was more accurate and aided in change in management in 35% of the patients. Further prospective head to head comparative trials are required to establish the role in initial evaluation and follow up pf BC.
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