Lymphocytic Variant Hypereosinophilic Syndrome: Case Series From a Tertiary Referral Center in Canada
Bibliographic record
Abstract
ABSTRACT Background Lymphocytic variant hypereosinophilic syndrome (L‐HES) is a rare disorder characterized by persistent eosinophilia driven by aberrant T‐cell populations. Diagnosis remains challenging due to the lack of standardized diagnostic criteria. Methods We retrospectively analyzed 18 patients diagnosed with L‐HES between 2016 and 2023. Comprehensive flow cytometry was performed on peripheral blood samples. Results Nine patients demonstrated the classic sCD3 − CD4 + CD5 + CD2 + CD45RO + CD45RA − immunophenotype, ranging from 0.6% to 70% of total lymphocytes. Two patients showed variant sCD3 − CD4 + phenotypes, five had expanded (> 10%) sCD3 + CD4 + CD7 − T‐cells, and two displayed aberrant CD8 + T‐LGL populations. Clonality was established in all patients with nonclassic phenotypes by molecular TCR testing or based on uniform TRBC1. We assessed a serial gating strategy to quantify the classic L‐HES phenotype and found this to be highly sensitive and specific with an estimated limit of detection of 0.06% of lymphocytes. Using this strategy, we identified decreased but detectable abnormal T‐cells in all classic phenotype patients reassessed posttreatment, down to as low as 0.3% of lymphocytes. The identification of T‐LGL phenotypes with eosinophilia is a novel finding. Conclusion Our study highlights the diverse immunophenotypic spectrum of L‐HES, emphasizing the importance of comprehensive flow cytometry analysis for accurate diagnosis.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
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 teacher head, 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".