Exceptional outcome: CD-30-positive relapsed enteropathy-associated T-cell lymphoma (EATL) treated with brentuximab vedotin alone, showing significant improvement in weight and albumin levels in Type II refractory coeliac disease: a case report
Introduction: Enteropathy-associated T-cell lymphoma (EATL) is the neoplastic transformation of intraepithelial T lymphocytes emerging in coeliac patients unresponsive to a gluten-free diet.1 Type 2 refractory coeliac disease (RCD II) is a precursor of EATL; therefore, RCD II has been added to the I...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2025-07-01
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| Series: | Clinical Medicine |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1470211825001162 |
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| Summary: | Introduction: Enteropathy-associated T-cell lymphoma (EATL) is the neoplastic transformation of intraepithelial T lymphocytes emerging in coeliac patients unresponsive to a gluten-free diet.1 Type 2 refractory coeliac disease (RCD II) is a precursor of EATL; therefore, RCD II has been added to the International Consensus Classification of mature T cell tumors.2 Given the significant morbidity related to EATL, the name has been modified from lymphoproliferative disorder to lymphoma as per WHO-HAEM5 classification.3 EATL has a very poor prognosis, with a median survival of 7 months.4 Reported 5‐year survival in the RCD II group was only 8% after developing EATL, with 79% relapsing within 1 to 60 months of diagnosis.5,6 Case description: A 44-year-old man presented to the gastroenterologists in 2017 and was diagnosed with type II refractory coeliac disease, for which he had 3 cycles of cladribine. The chemotherapy was completed in April 2018, with partial response but persistent clonal TCR rearrangement. He had 3 unsuccessful attempts at peripheral stem cell harvesting. He then presented with a 3-month history of falling albumin and worsening diarrhoea indicative of enteropathy progression in 2019. Capsular endoscopy showed significant deterioration in small bowel appearances, with concern for possible progression to EATL. A computed tomography (CT) scan confirmed diffuse ulcerative enteropathy of the jejunum with mesenteric nodes. A CD30-positive T cell lymphoma was diagnosed on biopsy from a right groin node. As the biopsy demonstrated CD30 expression, the patient was treated with brentuximab vedotin (BV)-cyclophosphamide, doxorubicin, prednisolone (CHP) as per the Echelon-2 trial.7 He struggled to tolerate the CHP and, therefore, for the last 2 cycles, he received single-agent BV. His end-of-treatment CT scan showed no evidence of lymphoma, but bowel wall appeared thicker and more dilated compared with a previous scan. He then re-presented with pain, nausea, diarrhoea and weight loss suggestive of relapsed disease. Given that retreatment with BV is NHSE commissioned,8 he was subsequently treated with BV monotherapy and had an excellent clinical response (Fig 1). Conclusion: BV is an antibody–drug conjugate directed against CD30 antigen, recommended as an option for treatment of relapsed anaplastic large-cell lymphomas (ALCL)8; as described, our patient was diagnosed with CD30-positive EATL with anaplastic morphology. Despite very poor prognosis of this disease, our patient had an excellent remission with BV for relapsed EATL with very good improvement in his weight and albumin level, maintaining a high quality of life. Malnutrition is central in the poor prognosis associated with RCDII9 and, in our case, the use of BV improved nutrition and albumin levels playing an important role in his survival. Although there are no standardised treatment protocols for this rare disease,10 this case illustrates an area of research and novel treatment delivery, including the role of BV in managing relapsed EATL. |
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| ISSN: | 1470-2118 |