Case 1
A 69-year-old female was admitted with a 6-month history of episodic fevers, 50-lb weight loss, anemia, and cognitive decline. Prior to admission, she had undergone extensive workup for cognitive decline, including MRI brain, EEG, and CSF analysis for viral, bacterial, fungal, and tuberculous infections; no cause of her symptoms could be identified. Her fevers had been evaluated with a comprehensive rheumatological workup, which was inconclusive. Imaging of the chest, abdomen, and pelvis did not show any occult malignancies. Peripheral blood flow cytometry and bone marrow biopsy was unremarkable. Pertinent labs on presentation include WBC 10.3 x 109/L, hemoglobin 9.6g/dL, platelets 157 x 109/L, total bilirubin 0.6mg/dL, ferritin 1432 ng/mL, LDH 404 IU/L, ESR 53 mm/hr, INR 1.5. Given her non-focal symptoms and lack of a definitive infectious or malignant etiology, intravascular lymphoma was suspected. Random deep skin biopsies were taken and showed several dilated blood vessels with sparse collections of large atypical lymphoid cells with irregular nuclei, prominent nucleoli, and moderately abundant cytoplasm. Cells were positive for CD20 and MUM1, and negative for CD10, BCL-2, and BCL-6. The patient urgently received one cycle of dose-reduced R-EPOCH (dose level -1) with GCSF support, which resulted in significant cognitive improvement over the next few weeks. The treatment course was complicated by neutropenic fever, recurrent hospital admissions, and functional decline. It was recommended that therapy be switched to mini-R-CHOP, but the patient’s poor ECOG status allowed single-agent rituximab therapy only. Follow-up PET/CT showed no evidence of disease. The decision was made to stop therapy as single-agent rituximab was considered insufficient for maintaining remission. The patient was placed on observation for the recurrence of signs and symptoms. She was disease-free at the time of the last contact with the system.