Post-transplant lymphoproliferative disorders (PTLDs) are lymphoid or plasmacytic proliferations ranging from polyclonal reactive proliferations to overt lymphomas that develop as consequence of immunosuppression in recipients of solid organ transplantation (SOT) or allogeneic bone marrow/hematopoietic stem cell transplantation. Immunosuppression and Epstein–Barr virus (EBV) infection are known risk factors for PTLD. Patients with documented histopathologic diagnosis of primary PTLD at our institution between January 2000 and October 2019 were studied. Sixty-six patients with PTLD following SOT were followed for a median of 9.0 years. The overall median time from transplant to PTLD diagnosis was 5.5 years, with infant transplants showing the longest time to diagnosis at 12.0 years, compared to pediatric and adolescent transplants at 4.0 years and adult transplants at 4.5 years. The median overall survival (OS) was 19.0 years. In the monomorphic diffuse large B-cell (M-DLBCL-PTLD) subtype, median OS was 10.7 years, while median OS for polymorphic subtype was not yet reached. There was no significant difference in OS in patients with M-DLBCL-PTLD stratified by quantitative EBV viral load over and under 100,000 copies/mL at time of diagnosis, although there was a trend towards worse prognosis in those with higher copies.
Patients with B-lymphoid malignancies have been consistently identified as a population at high risk of severe COVID-19. Whether this is exclusively due to cancer-related deficits in humoral and cellular immunity, or whether risk of severe COVID-19 is increased by anticancer therapy, is uncertain. Using data derived from the COVID-19 and Cancer Consortium (CCC19), we show that patients treated for B-lymphoid malignancies have an increased risk of severe COVID-19 compared with control populations of patients with non–B-lymphoid malignancies. Among patients with B-lymphoid malignancies, those who received anticancer therapy within 12 months of COVID-19 diagnosis experienced increased COVID-19 severity compared with patients with non–recently treated B-lymphoid malignancies, after adjustment for cancer status and several other prognostic factors. Our findings suggest that patients recently treated for a B-lymphoid malignancy are at uniquely high risk for severe COVID-19. Significance: Our study suggests that recent therapy for a B-lymphoid malignancy is an independent risk factor for COVID-19 severity. These findings provide rationale to develop mitigation strategies targeted at the uniquely high-risk population of patients with recently treated B-lymphoid malignancies. This article is highlighted in the In This Issue feature, p. 171
1 December 2022 in Journal of Gastrointestinal Oncology
Case Report Concurrent Epstein Barr virus-associated smooth muscle tumor and myeloid sarcoma of the liver and acute myeloid leukemia in a patient post kidney transplant: a case report and review of the literature Victor Wang, Kimrey Van Perre, Lu Pu, Yan Liu, Jun Wang, Evelyn Choo, Justin Moyers, Huynh Cao, Eric Lau
6 April 2018 in Journal of Gastrointestinal Cancer
Background/Objectives: Multicentric Castleman Disease (MCD) is a rare lymphoproliferative disorder that can mimic IgG4-related disease (IgG4-RD), particularly in patients presenting with elevated serum IgG4. Accurate diagnosis is crucial given differing treatments and prognoses. Case Presentation: We describe a 76-year-old male with fever, lymphadenopathy, and elevated inflammatory markers. Labs revealed an elevated IgG4 of 133 mg/dL and total IgG of 1410 mg/dL, yielding an IgG4/IgG ratio of 9.43%. Lymph node biopsy showed nodular sclerosing classical Hodgkin lymphoma, for which he received five cycles of A + AVD. Persistent symptoms, elevated IL-6, and HHV8 viremia prompted repeat biopsy, which demonstrated HHV8-positive MCD. Rituximab was initiated, which resulted in clinical and radiographic resolution. Methods: We performed a systematic review of the English-language literature from 2000 to 2025, identifying 23 studies that contained MCD cases with individual-level serum IgG4 and IgG data. A total of 36 unique cases were included. Results: The mean IgG4/IgG ratio was 14.61%, which is substantially lower than ratios typically seen in IgG4-RD. To our knowledge, our case is the only reported instance of HHV8-associated MCD with elevated IgG4. Conclusions: A mildly elevated IgG4/IgG ratio may favor the diagnosis of MCD over IgG4-RD. Serum IgG4 and total IgG should be considered when suspecting Castleman Disease.