HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS: TWO INSTRUCTIVE CASES
Bibliographic record
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is rare in adults. Two exemplary cases are presented here illustrating the clinical features, treatment and natural history of this aggressive and often fatal disease. A 21 year old man presented with jaundice and fever and was found to have CMV-triggered HLH. He responded well to the HLH-2004 protocol and remains in remission. A 45 year old woman presented with erythroderma and multi-organ failure and was found to have HLH associated with an abnormal T-cell population and low-titre EBV. She died despite etoposide-based therapy. HLH should be considered in patients with persistent unexplained inflammation, cytopenias, hepatitis, and coagulopathy. Serum ferritin is a useful screening test and hyperferritinemia > 10 000 μg/L is thought to be specific for hemophagocytosis. Tissue biopsy (bone marrow, lymph node or liver) to confirm hemophagocytosis is desirable, but is often a late finding and is not necessary for the diagnosis of HLH. Once the diagnosis is confirmed, rapid initiation of chemo-immunotherapy is required to induce remission.
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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 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.004 | 0.002 |
| 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".