Neoplastic PTLD. Case 4.

Posttransplant Hodgkin's Disease

This young male received a liver transplant for relief of sclerosing cholangitis. His underlying disease was a complication of Crohn’s disease which had been diagnosed 8 years earlier. His family history was positive for ulcerative colitis, Hodgkin’s disease, leukemia, and diabetes in close family members. Following uncomplicated liver transplant, he was discharged under Cyclosporine/steroid immunosuppression. His only complication was a tooth abscess two months posttransplant. Five months after transplant he complained of throbbing flank pain, and developed fever to 103F without chills, anorexia and fatigue. Ultrasonography revealed paraaortic nodes in the distal aorta and pelvis, with no adenopathy elsewhere. Urine was positive for CMV. A lymph node biopsy was consistent with mixed cellularity Hodgkin’s disease. A trial of reduced imunosuppression was initiated. The patient returned to his home state and was apparently well for another 4 years, at which time recurrence of a histologically similar tumor occurred. Few details of this hospitalization are available at present, but he apparently received chemotherapy for Hodgkin’s disease. The patient expired 3.5 years following the original tumor diagnosis. The slide series is taken from the original node biopsy and shows effacement by a mixed cell infiltrate consistent with Hodgkin’s disease. The infiltrate extended beyond the capsule, and in some areas residual node elements were identifiable. Reed-Sternberg and Hodgkin cells were easily identified. Focal necrosis was observed (the patient had not received chemotherapy prior to this biopsy). Eosinophils were focally prominent and an example is given here. The large atypical cells were positive for EBV by the EBER stain. These cells were also among the replicating cells according to immunostain for cell cycle-associated antigens. Molecular clonal studies were not available.

Posttransplant Hodgkin’s disease is unusual in comparison to posttransplant non-Hodgkin’s lymphoma. The family history of Hodgkin’s disease is of interest in this case and it would also be of interest to know if other transplant patients with Hodgkin’s disease had a positive family history. In our experience it is more common to see PTLDs with "Hodgkin’s-like" features, rather than frank Hodgkin’s disease. These "Hodgkin's-like" tumors are also characterized by a minor population of neoplastic-appearing large cells which have an appearance and phenotype similar or identical to Reed-Sternberg cells. These cells also contain EBV. The background cells are lymphoid, many with an "activated" appearance. The few tumors of this type have contained monoclonal B cells, which likely correspond to the large cell population.

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