Infection as Trigger for Congenital Thrombotic Thrombocytopenic Purpura in an Adult Patient
Bibliographic record
Abstract
Congenital thrombotic thrombocytopenic purpura (cTTP) is an inherited disease that is sometimes fatal in early childhood. cTTP is similar to idiopathic thrombotic thrombocytopenic purpura (iTTP); both are characterized by varying levels of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and end-organ damage secondary to occlusion of the microvasculature. cTTP is caused by a partial or total deficiency or loss of function of ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13). We report the case of a 33-year-old woman who was mistakenly diagnosed with primary immune thrombocytopenia (ITP) during childhood. The patient was referred to our center with dyspnea, fatigue, fever, and jaundice with no clinical bleeding. Laboratory features were compatible with MAHA; ADAMTS-13 activity was at 0%, with negativity for ADAMTS-13 antibodies. We concluded the final diagnosis was cTTP. The triggering factor identified for MAHA was a double infection: central venous catheter bacterial infection and atypical pneumonia. After 7 days of treatment with antibiotics and ongoing total plasma exchange (TPE), the patient responded favorably. Our patient received fresh frozen plasma (FFP) infusion once every 2 weeks, and prophylactic voriconazole remained under control at the time of writing. As demonstrated in this case, effective treatment of the trigger cause helps reduce the need for continuous FFP exposure and controls the MAHA.
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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 | 0.003 |
| 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) | 0.004 | 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".