Systemic Epstein-Barr Virus-Positive T-Cell Lymphoma of Childhood Associated With t(1;22)(p22;q11.2) Mutation
Bibliographic record
Abstract
Systemic Epstein-Barr virus-positive (EBV + ) T-cell lymphoma (TCL) of childhood is an uncommon TCL that occurs secondary to an acute or chronic EBV infection. The disorder is characterized by the monoclonal expansion of EBV + T cells driven by an increased immune response and defect in regulatory pathways. Thus, systemic EBV + TCL of childhood is frequently associated with a hyperinflammatory state, hemophagocytic lymphohistiocytosis (HLH) syndrome, and exhibits a fulminant clinical course with poor outcomes. Additionally, genetic alterations at specific chromosome loci, such as chromosome 22q11.2, are hypothesized to increase the chances of carcinogenic transformation and increase the risk of non-Hodgkin lymphoma later in life. Chemotherapy, immunotherapy, and allogenic stem cell transplants are treatment options with varying degrees of success. In this report, we describe a case of a 21-year-old male with a primary acute EBV infection that led to HLH syndrome. He was ultimately diagnosed with systemic EBV + TCL of childhood. Despite treatment chemotherapy, the patient passed before an allogenic stem cell transplant could be performed. We explore the clinicopathological features of his disease and a possible new oncogenic locus at the t(1;22)(p22;q11.2) breakpoint. Our case underscores the importance of retaining a wide differential diagnosis, including unusual presentations of systemic EBV + TCL of childhood, when presented with an adult case of HLH. It also highlights a possible new genetic locus associated with immunological malignancies that warrants further study. J Hematol. 2024;13(5):229-237 doi: https://doi.org/10.14740/jh1284
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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 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".