P‐IG‐30 | Management of a Sickle Cell Disease Patient with Multiple Alloantibodies in Preparation for a Bone Marrow Transplant
Bibliographic record
Abstract
Hematopoietic stem cell transplant (HSCT) is currently the only curative option for patients with sickle cell disease (SCD). Alloimmunization in this population is frequent and can complicate transfusion management during HSCT period. The transfusion management strategies for a pediatric SCD patient with severe clinical phenotype, and history of multiple alloantibodies (nine) and hyperhemolysis syndrome who successfully underwent HSCT are described. The 12-year-old patient underwent haploidentical HSCT following myeloablative conditioning. He received daratumumab, rituximab, bortezomib and had 5 plasma exchanges for desensitization to donor specific anti-HLA antibodies and for the erythrocytes alloimmunization. The patient is known for an anti-e, despite the presence of an RHCE*01.01 allele which predicts a C−, c+, E-, weak e+ phenotype. Because of the patient significant alloimmunization history (anti-M, S, C, e [variant], K, Fy3, Fya, Jkb, s [variant], and probable auto anti-N), it was decided to match units for the RHCE*01.01 allele and all donors matching the patient extended phenotype were targeted for RHCE genotyping. Donors homozygous or heterozygous for RHCE*01.01 were then selected for compatibility analyses and ranked based on strength of reactions. Discordance between zygosity and strength of reactions were observed, as the most compatible donors were heterozygous for RHCE*01.01. Various strategies were also implemented to limit donor exposure. Notably, transfusion decisions were based on anemia tolerance. In total, the patient received 7 red blood cell units from 2 different donors (heterozygote RHCE*01/RHCE*01.01) during HSCT process without transfusion reaction or development of alloantibodies (see Table 1). No reaction during compatibility analyses were observed for donor 1 whereas weak reactions with unabsorbed serum were detected for donor 2, but none with allo-adsorbed serum. Five months post-HSCT, hemoglobin level is stable above 130 g/L and chimerism is 100%. TABLE 1. Transfusion history during HSCT. HSCT, hematopoietic stem cell transplant.
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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 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| 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".