A 59-year-old female presented to our institution with a four-week history of malaise, myalgia, chills, and unintentional weight loss. She also endorsed rhinorrhea and a non-productive cough over this time. She had no medical history or prescription medications, although she took daily supplements including Vitamin C, Vitamin D, zinc, garlic, and a multivitamin. She used marijuana intermittently but did not smoke tobacco or use any other substances. Upon presentation, she was afebrile with an oxygen saturation of 96% on room air and otherwise stable vital signs. Her respiratory, cardiac, and abdominal examinations were unremarkable.
Initial investigations were notable for a peripheral lymphocyte count of 52.2 x 109/L and flow cytometry showing 70% involvement of an immunophenotypically abnormal monoclonal plasma cell population. A bone marrow biopsy performed on Day 4 of her hospital admission confirmed a diagnosis of plasma cell leukemia. Of note, CT chest performed upon admission to hospital did not demonstrate any airspace disease.
On Day 8, she began therapy with bortezomib 1.3 mg/m2 weekly, dexamethasone 40 mg weekly, and lenalidomide daily. She was afebrile and her oxygen saturation was 95% on room air. The next day, her cough worsened significantly from earlier in the admission, and the sputum contained small volumes of dark blood. The hemoptysis and severe cough persisted on Day 10; she remained afebrile with an oxygen saturation of 96% on room air. A CT scan of the chest demonstrated bilateral patchy ground glass opacities with regions of crazy paving and new small bilateral pleural effusions (Fig. 1).
Bronchoscopy was performed on Day 11. Sequential bronchoalveolar lavage demonstrated increasingly hemorrhagic returns. Microbiologic testing for bacterial and fungal cultures, COVID-19, influenza, RSV, TB, PJP, and galactomannan was negative, and cytology did not demonstrate any malignant cells. A workup for alveolar hemorrhage was initiated. Anti-nuclear antibodies, rheumatoid factor, anti-cyclic citrullinated peptide (anti-CCP), extractable nuclear antigen (ENA) panel, anti-nuclear cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), complements C3 and C4, cryoglobulin, anti-cardiolipin antibodies, and lupus anticoagulant testing were all within normal limits. CRP was 82.1 mg/L (2.9 mg/L on admission).
The patient remained clinically stable on room air, with no further hemoptysis after Day 12 of her admission and with gradual improvement in her cough. Therefore, she was not immediately initiated on corticosteroids, aside from the 40 mg of weekly dexamethasone in her chemotherapeutic regimen. However, she became febrile on Day 13 and on Day 14, out of concern that the fevers may be a delayed manifestation of a bortezomib-related reaction, she was started on prednisone 70 mg daily. A septic workup was negative, and the fevers subsided.
A repeat CT scan of the chest on Day 17 of her admission showed near complete resolution of the ground glass opacities, with some residual mosaicism in the upper lung zones, and a decrease in the size of the effusions. She was discharged home shortly afterward and continued therapy with lenalidomide and a tapering dose of steroids; our service advised that she not be rechallenged with bortezomib. She has not had any recurrence of her respiratory symptoms.