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A Diffuse Medullary Hypercaptation With No Bone Lesion

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 71-year-old man had a 3-year history of a recurrent seronegative autoimmune constrictive pericarditis and pleural effusions, which were treated with pericardiectomy and prednisone and azathioprine. The pleural fluid was transudative and sterile, and the pericardium contained focal lymphoplasmacytic aggregates. Attempts to taper immunosuppressive drugs failed because of a recrudescence of the inflammatory syndrome and pericardial effusion. The patient was admitted with fever of unknown origin with a surge of the inflammatory syndrome and pancytopenia. He reported night sweats beginning 3 weeks prior associated with fever and unintentional weight loss. No lymphadenopathy was noted.

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B. Bone marrow–liver–spleen type of large B-cell lymphoma

Fluorodeoxyglucose/PET-CT imaging results (Figure, A and B) showed diffuse medullary hypercaptation of the entire skeleton with splenic activity without lymph node involvement. Bone-marrow biopsy results (Figure, C and D) showed hypercellular marrow with a diffuse interstitial infiltrate of large, centroblasticlike lymphoid cells and no features suggestive of plasmacytic or secretory differentiation. These cells were CD20+, CD5, and CD30 and accounted for approximately 60% of the global cellularity. The large B cells were CD10, BCL6+, and MUM-1+, a non-germinal center–like phenotype according to the Hans algorithm. Furthermore, B cells were BCL2+ and negative for MYC expression, and fluorescence in situ hybridization studies showed no rearrangements of the BCL2, BCL6, or MYC genes. Although immunostains for κ and λ light chains highlighted a small population of reactive plasma cells distributed in the marrow spaces, they did not produce specific staining in the lymphoma cells. Residual trilinear hematopoiesis was reduced, with no excess of blasts, and no evidence was found to support a diagnosis of hemophagocytic lymphohistiocytosis. The lymphoma cells were negative for Epstein-Barr virus and human herpesvirus 8.

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Article Information

Corresponding Author: Michel Obeid, MD, PhD, Centre d’Immunothérapie et de Vaccinologie, Centre Hospitalier Universitaire Vaudois, Rue du Bugnon 17, 1011 Lausanne, Switzerland (michel.obeid@chuv.ch).

Published Online: January 16, 2020. doi:10.1001/jamaoncol.2019.4655

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s wife for granting permission to publish this information.

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