Stepwise Treatment for TAFRO Syndrome
Bibliographic record
Abstract
TAFRO syndrome, a rapidly progressive and fatal disease, is rare, and its etiology remains unknown. It is characterized by thrombocytopenia, anasarca (edema, pleural effusion, and ascites), fever, reticulin fibrosis (or renal insufficiency), and organomegaly with Castleman disease (CD)-like histological features in the lymph nodes. CD is a rare, indolent, lymphoproliferative disorder with no established curative strategies. Most idiopathic multicentric CD cases are controlled with anti-interleukin (IL)-6 therapy (tocilizumab and siltuximab) and/or rituximab. However, it is unclear whether these therapies can be directly applied to treat TAFRO syndrome. Here, we describe stepwise immunotherapy (rituximab induction therapy and cyclosporine maintenance therapy) for two cases of steroid-refractory TAFRO syndrome. A 32-year-old man visited a local hospital with sudden onset of fever and epigastralgia. The diagnosis of TAFRO syndrome was established based on the diagnostic criteria. After rituximab administration, C-reactive protein and IL-6 levels were normalized. However, the ascites persisted, with increased resistance to rituximab. Tocilizumab was also ineffective; therefore, cyclosporine was administered. After the initiation of cyclosporine treatment, the ascites decreased and ultimately disappeared. Twelve months after immunotherapy, the patient remained asymptomatic under cyclosporine maintenance therapy. Similar stepwise immunosuppressive therapy was administered to a 72-year-old man with TAFRO syndrome complicated by renal failure. After rituximab infusion, C-reactive protein was decreased. Although methylprednisolone, rituximab, tocilizumab, and cyclosporine were administered, other laboratory data and clinical symptoms remained unchanged. His level of consciousness subsequently deteriorated due to herpes zoster encephalitis, and he died. We consider the combination of rituximab induction therapy and cyclosporine maintenance therapy to be effective for TAFRO syndrome if initiated at an early stage.
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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 | 0.001 |
| 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) | 0.001 | 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 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".