Post-Transplant Lymphoproliferative Disorder Manifesting as Lymphoplasmacytic Lymphoma Accompanying With Hemophagocytic Lymphohistiocytosis
Bibliographic record
Abstract
Post-transplant lymphoproliferative disorder (PTLD) is a potentially life-threatening complication, often associated with Epstein-Barr virus (EBV) in the early period after hematopoietic stem cell transplantation (HSCT). Clinical manifestations range from localized to disseminated disease. The cornerstone of therapy is the reduction of immunosuppression and/or immunochemotherapy. We report a 39-year-old female who developed PTLD presenting as lymphoplasmacytic lymphoma (LPL) associated with hemophagocytic lymphohistiocytosis (HLH). Diagnostic evaluation was blurred by features of severe hepatic acute graft-versus-host disease (GVHD). An initial treatment consisted of high-dose steroids, but it failed. As second-line treatment, ruxolitinib and mycophenolate mofetil were administered, but they were ineffective, and the patient's condition worsened. Further detailed evaluation revealed the presence of monoclonal protein immunoglobulin G (IgG) lambda and bone marrow infiltration by clonal plasmacytoid B lymphocytes. The HLH criteria were also met. Immunosuppression was discontinued, and dexamethasone with rituximab was initiated, but no response was observed. The patient eventually died from multiple organ failure. The learning points from this case emphasize that HLH in the context of PTLD remains underreported, with few cases documented in the literature. Studies indicate that EBV plays a central role in pathogenesis, often presenting with systemic inflammation and immune dysregulation. Diagnostic challenges arise due to overlapping clinical features with other post-transplant complications. Treatment strategies vary but often involve balancing immunosuppression reduction and chemotherapy, with rituximab being a cornerstone for EBV-driven cases. This case underscores the necessity of early recognition to mitigate severe outcomes.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".