Clinical Course and Pathological Findings in a Series of Patients with Aggressive Natural Killer (NK) Cell Leukemia
Bibliographic record
Abstract
Abstract Abstract 4823 Background: NK cell leukemia is a rare, malignant proliferation of NK cells associated with Epstein Barr Virus (EBV). The World Health Organization (WHO) defines NK cell leukemia based on a blood film showing pancytopenia, and circulating leukemic cells, which are positive for CD2, cytoplasmic CD3, CD56, and cytotoxic molecules. The neoplastic cells may have a wide range of appearances, including cells that resemble large granular lymphocytes, highly atypical cells with abundant cytoplasm, and azurophilic granules. Reported cytogenetic abnormalities have included 6q deletion, 11 q deletion and losses of 7p and 17p. Previous publications in the medical literature have been limited to case reports, mostly from Asian centers. Methods: We conducted a retrospective chart review of all aggressive NK cell leukemia diagnoses at an academic medical center, from 2008–2012. Results: We identified 4 cases of aggressive NK cell leukemia. The median age at diagnosis was 57 years. 3 out of 4 cases were male, and 3 of 4 were associated with EBV infection. All patients presented clinically with cytopenias, one patient had an elevated white blood cell count, 31 × 10 9per liter with the others presenting with pancytopenia. All had constitutional symptoms (including fever and night sweats) at diagnosis. 2/4 cases had extramedullary involvement, including splenomegaly and lymphadenopathy. One patient presented with hemophagocytic lymphohistiocytosis. 2/4 patients had CNS involvement. All had a large proportion of circulating neoplastic cells (12–80%). The blood film examination in all cases demonstrated large, pleomorphic neoplastic cells. Most (3/4) had distinct nucleoli and 1/4 had large azurophilic granules in the cytoplasm. The immunohistochemistry profile showed positivity for cytoplasmic CD3, CD56 and CD7 in all cases. 3/4 cases were positive for CD11c. 2/4 patients had unique complex karyotypes including chromosome 3p duplication, deletion of 16q, addition 1p, trisomy 7, trisomy 8, isochrome 17q and near tetraploid karyotype. All patients received treatment with various chemotherapeutic protocols; however none received radiation therapy, or stem cell transplantation. 2/4 cases achieved a short remission. All patients had poor clinical outcomes with median survival of 12 months (range 2–19 months), which is consistent with prior reports. Conclusion: This case series serves to confirm the typical clinical presentation, and poor prognostic outlook, of patients with aggressive NK cell leukemia. This rare malignancy was previously described only in individual case reports. In our patient population, we noted complex, novel cytogenetic findings. Our study reaffirms the need for new therapeutic modalities to improve the poor prognosis for this disease. Disclosures: No relevant conflicts of interest to declare.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 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".