Gluten Gambit: A Case of Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
Bibliographic record
Abstract
Purpose: Hemophagocytic lymphohistiocytic syndrome (HLH) is an uncommon syndrome characterized by overactive histocytes induced by disorders such as T-cell lymphoma. We describe a rare case of HLH in enteropathy-associated T-cell lymphoma (EATL) and refractory celiac disease (RCD). Methods: A 70-year-old woman with a 4-year history of celiac disease was transferred to our tertiary care hospital from a community hospital for RCD unresponsive to strict gluten-free diet and one-month of prednisone therapy. She initially presented with a 4-month history of 25 lbs weight loss and diarrhea. Abdominal CT demonstrated only small bowel inflammation. Multiple esophagogastroduodenoscopies and colonoscopies were optically normal. She was diagnosed with RCD type II based on duodenal and jejunal biopsies, showing severe villous blunting and intraepithelial lymphoplasmacytic infiltration. Upon arrival to our institution, the patient was tachycardiac, hypotensive, with ongoing melena and hematochezia. She had intermittent gastrointestinal (GI) bleeding prior to her transfer without an obvious source identified. She also experienced intermittent fevers and was treated empirically with broad-spectrum antibiotics. She was diagnosed with HLH after developing pancytopenia, low fibrinogen, elevated ferritin and hemophagocytosis on her bone marrow biopsy. She had no response to HLH treatment with dexamethasone, cyclosporin, anakinra, etoposide or intravenous immunoglobulin. Given her clinical picture suggested EATL, her previous biopsies were reviewed by a lymphoma pathologist. He revised the final diagnosis to type I EATL based on the high proportion of large T cells, infiltrating the lamina propria, and the abnormal T cell marker profile (positive CD3, 7, 30; negative CD5, 56). Unfortunately, the patient subsequently died from a massive small bowel GI bleed. Conclusion: To our knowledge, this is the first known case of HLH associated with localized EATL and RCD. Only 7 cases of HLH with EATL have been reported but none previously had documented RCD. We aim to highlight HLH as a rare but important condition that gastroenterologists must be aware of in their patients with RCD and EATL. Although, in all previous reported cases, HLH presented in the context of advanced EATL with metastasis, this case illustrates the importance of prompt referral as it is the first HLH case that presented with non-advanced EATL and facilitated early HLH therapy. Lastly, this case serves as a reminder that when suspicion is high and EATL is not seen on imaging, the clinician must remember that EATL is a histological diagnosis, requiring tissue and experienced pathology opinion.
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 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 | 0.002 |
| 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 | 0.002 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 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".