Treatment response in enteropathy associated T‐cell lymphoma; survival in a large multicenter cohort

Abstract

Enteropathy‐associated T‐cell lymphoma (EATL) is a T‐cell Non‐Hodgkin Lymphoma which is highly associated with celiac disease. The prognosis of EATL has been considered poor and there are no standardized treatment protocols. Here, we evaluate treatment response and survival of EATL patients in a large multicenter cohort. A total of 61 patients diagnosed with EATL were analyzed. Various treatment regimens were applied in EATL during the past fifteen years including either monotherapy consisting of chemotherapy or resection, or combination therapy with both aforementioned regimens whether or not combined with stem‐cell transplantation (SCT). Overall, 50/61 patients (82%) died after a median of 7.4 months. One‐ and five‐year overall survival was 40 and 11%, respectively. Median follow‐up in the survivors was 26 months. Patients treated with the most aggressive treatment, that is, resection, chemotherapy and autologous SCT, showed the most favourable outcome with complete remission in all patients, the lowest relapse rate and one‐ and five‐year overall survival of 100 and 33%, respectively, although overall survival in this group was not significantly better as compared to patients treated with surgery and chemotherapy. This study indicates that combination treatment is superior compared to monotherapy. Whether or not consolidation therapy with autologous SCT may improve survival needs to be substantiated in a larger randomized international trial. Am. J. Hematol. 90:493–498, 2015. © 2015 Wiley Periodicals, Inc.

Enteropathy-Associated T-Cell Lymphoma: Improving Treatment Strategies

“…These studies showed a lower amount of patients failing the high-dose chemotherapy due to disease progression as compared to anthracycline-based chemotherapy. Moreover, patients treated with high-dose chemotherapy and autologous SCT (auSCT) had higher remission rates than those treated with standard induction therapy including CHOP and additional auSCT [9,10,11]. However, as expected, there was a higher incidence of neutropenic fever and sepsis compared with standard induction chemotherapy.…”

“…Overall, patients treated with combination therapy consisting of surgical debulking and subsequent standard induction chemotherapy show a significantly better outcome than those treated with standard induction chemotherapy or surgery alone [7,8,9,14]. This stresses the importance of early diagnosis of EATL and extensive nutritional assessment to optimize nutritional status at diagnosis and during therapy.…”

“…As shown in different independent studies, patients with secondary EATL have a less favourable outcome than EATL patients without a prior diagnosis of CD [8,9,15]. Within the group of secondary EATL, patients who suffer from refractoriness to the gluten-free diet (RCDII) even have a worse prognosis.…”

“…Standard induction chemotherapy regimens applied in EATL are usually anthracycline based consisting of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) in most cases [8,10,12]. Five-year survival percentages following surgical debulking and standard induction chemotherapy vary from 8 to 28% [7,8,9,14]. The most common reason for withholding chemotherapy after surgical debulking is a poor condition of the patient after surgery.…”

“…Besides the aggressive and often chemotherapy-refractory nature of the T-cell lymphoma itself, the deprived nutritional status of most EATL patients at diagnosis seems to be an important factor which negatively affects clinical condition and therefore preventing the use of aggressive treatment strategies. This results in an extremely variable 5-year survival of EATL patients between 8 and 60% [5,6,7,8,9,10,11]. …”