A case of acquired angioedema associated with Waldenstrom’s macroglobulinemia treated with rituximab
Bibliographic record
Abstract
Acquired angioedema (AAE) is a rare disorder caused by acquired deficiency of C1 inhibitor (C1-INH), very often seen in association with lymphoproliferative diseases. The key elements of AAE are acquired deficiency of C1 inhibitor, hyperactivation of the classical pathway of complement, and recurrent angioedema symptoms. We report a case of Waldenstrom´s macroglobulinemia causing an acquired deficiency of C1 esterase inhibitor in a 40-year-old woman. She initially presented with an episode of angioedema followed by many episodes of abdominal distention associated with pain, vomiting, and diarrhea for 1.5 years. Work-up revealed low C1 esterase inhibitor levels, normal C3, and nonexistent C4. A diagnosis of acquired C1 esterase inhibitor deficiency was proposed at the time. However, it was also noted that her IgM was very elevated with an IgM monoclonal gammopathy with kappa light chains, and an enlarged spleen. Bone marrow biopsy and aspirate revealed clonal B-cells staining positively for CD20. She was diagnosed with Waldenstrom´s macroglobulinemia in association with C1 esterase inhibitor deficiency. Although the association is recognized, it is rare, and likely secondary to antibodies against C1 esterase inhibitor. After treatment with rituximab, cyclophosphamide, and prednisone, her paraprotein levels fell to normal range, and her autoimmune parameters normalized with significant clinical improvement. She has not had any further episodes of angioedema. She will continue on rituximab as maintenance therapy for 2 more years. Acquired deficiency of C1 esterase inhibitor is quite rare with just over 100 cases in the literature. Given that most cases are related to antibodies against C1 esterase inhibitor, rituximab may be the treatment of choice. However, treatment of the underlying lymphoproliferative disorder may need to be considered as well and could provide a more definitive treatment.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.004 | 0.002 |
| 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".