Gluten Gambit: A Case of Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease
Notice bibliographique
Résumé
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.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,001 | 0,001 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,002 | 0,001 |
| Études des sciences et des technologies | 0,002 | 0,001 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,001 | 0,002 |
| Intégrité de la recherche | 0,003 | 0,002 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».