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Record W3032161358 · doi:10.14740/jmc3478

Two Roads Diverge: Treatment Choice in Coexisting Severe Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria

2020· article· en· W3032161358 on OpenAlexvenueno aff
Aamina Shakir, Sunny Narula, Austin L. LaGrow, Sanober Nusrat

Bibliographic record

VenueJournal of Medical Cases · 2020
Typearticle
Languageen
FieldImmunology and Microbiology
TopicComplement system in diseases
Canadian institutionsnot available
Fundersnot available
KeywordsMedicineEculizumabParoxysmal nocturnal hemoglobinuriaPancytopeniaBone marrow failureAplastic anemiaNeutropeniaBone marrowHemoglobinuriaMyelodysplastic syndromesInternal medicineGastroenterologyAnemiaPediatricsChemotherapySurgeryImmunologyHaematopoiesisStem cellImmune system

Abstract

fetched live from OpenAlex

Aplastic anemia (AA) is a bone marrow failure syndrome of pancytopenia due to impaired hematopoiesis. It is strongly associated with paroxysmal nocturnal hemoglobinuria (PNH). Each condition can cause the other, or occur simultaneously. There are no guidelines for treating concomitant AA and PNH; immunosuppressive therapy (IST) or hematopoietic stem cell therapy (HSCT) is first-line for the former, and eculizumab is first-line for the latter. New studies suggest that treating AA/PNH together versus sequentially should depend on AA severity. We report the case of a previously healthy male (31-year-old, Nigerian immigrant) who developed jaundice, scleral icterus, easy fatigability, and epistaxis. He was diagnosed with AA on bone marrow biopsy and with PNH on flow cytometry. He initially underwent chemotherapy due to increased infection risk with eculizumab in a neutropenic patient; however, he showed minimal response and thus began eculizumab pending allogeneic stem cell transplant. There are no guidelines for treating patients with both AA and PNH, and clinical decision making is generally individualized based on disease severity. Only one prior publication reported simultaneous treatment with eculizumab and chemotherapy, due to stated concern for pancytopenia, especially neutropenia, being the most immediate cause of morbidity/mortality. This demonstrates the individualized decisions that must be made when treating simultaneous PNH and AA, and the importance of PNH/severe AA patients as a separate subpopulation.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.002
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesInsufficient payload (model declined to judge)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.309
Threshold uncertainty score0.999

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0020.000

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.

Opus teacher head0.043
GPT teacher head0.315
Teacher spread0.272 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

Study designObservational
Domainnot available
GenreEmpirical

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".

Quick stats

Citations4
Published2020
Admission routes1
Has abstractyes

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