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.