An Unusual Case of Extracavitary/Solid Variant Primary Effusion Lymphoma With Associated Hemophagocytic Lymphohistiocytosis
Bibliographic record
Abstract
Primary effusion lymphoma (PEL) is a rare, aggressive large B-cell lymphoma variant that is invariably associated with human herpesvirus 8 (HHV8), predominantly in human immunodeficiency virus (HIV)-infected patients, and its oncogenicity is often augmented by coinfection with Epstein-Barr virus. It typically presents as a serous effusion in body cavities without detectable solid tumors. The extracavitary variant of PEL may represent a diagnostic challenge. A 37-year-old man with HIV/acquired immunodeficiency syndrome (AIDS) was transferred to our hospital for evaluation of a mediastinal mass with associated clinically diagnosed hemophagocytic lymphohistiocytosis (HLH), fever, pancytopenia, hepatosplenomegaly, retroperitoneal lymphadenopathy, and wasting syndrome. Contrast-enhanced computed tomography showed a large soft tissue mass extending along the middle/posterior mediastinum into the left hilum and a large left pleural effusion. Endoscopic fine-needle biopsy of the lesion showed sheets of large pleomorphic lymphoma cells with prominent nucleoli and abundant cytoplasm. These cells were also seen on the cytospin smear of pleural fluid. Immunohistochemical stains showed lymphoma cells positive for CD3 (small subset), CD45, CD138, MUM-1, and HHV8 and negative for CD5, CD20, CD30, ALK1, AE1/3, and PAX-5. The lymphoma cells were also positive for Epstein-Barr virus-encoded RNA (EBER) (in situ hybridization). Solid masses in extracavitary PEL have been shown to involve lymph nodes and/or solid organs such as the gastrointestinal tract, lung, liver, spleen, and skin, with a similar phenotype as classic PEL except that they may express B-cell markers with lower expression of CD45 and/or aberrant coexpression of T-cell antigens. This case illustrates the unusual manifestation of PEL as a mediastinal mass with associated HLH.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.002 | 0.002 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.004 | 0.003 |
| Insufficient payload (model declined to judge) | 0.003 | 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".