Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges.

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as enteropathy associated T-cell lymphoma (EATL) type II, is a rare haematological malignancy with a difficult and delayed diagnosis. Symptoms can include abdominal pain, weight loss, and chronic diarrhoea. However, most patients are only diagnosed after complications, such as perforation or obstruction, have developed. There is no standard treatment for MEITL; most accepted regimens consist of surgical resection and anthracycline-based chemotherapy. Prognosis is poor with an approximate survival of less than a year. Even though other therapies, such as autologous stem cell transplant, has shown promising results, not all patients can tolerate this course of treatment especially if they are elderly, have several comorbidities or are malnourished. Innovative therapies that improve survival and can be used as an alternative for more intensive treatment are needed. We report the use of PEG-asparaginase along with conventional anthracycline therapy in a 70-year-old woman diagnosed with MEITL, who went into remission and survived for more than one year before succumbing to relapsed disease.

Patient age and number of apheresis days may predict development of secondary myelodysplastic syndrome and acute myelogenous leukemia after high-dose chemotherapy and autologous stem cell transplantation for lymphoma.

BACKGROUND: The goal of our study was to find predictors for the development of secondary myelodysplastic syndrome or acute myelogenous leukemia (s-MDS/AML) in patients with relapsed or refractory lymphoma who received high-dose chemotherapy and autologous stem cell transplantation (ASCT). STUDY DESIGN AND METHODS: We conducted a retrospective review of 295 patients with relapsed or refractory lymphoma who had undergone their first stem cell collection and ASCT. Patient, disease, and treatment characteristics were collected. The primary goal of this study was to analyze the association between the number of apheresis days needed to collect the requisite stem cell dose in addition to the previously described factors such as age, sex, number and type of prior chemotherapeutic regimens, disease type and status, and the risk of developing s-MDS/AML. RESULTS: Twenty-two patients of 295 were diagnosed with s-MDS/AML after a median follow-up of 62 months. Multivariate analysis using a classification and regression tree showed that the incidence of s-MDS/AML was lowest in patients who were not more than 55 years old at transplantation and in whom the target cell dose was collected in fewer than two apheresis sessions (5-year cumulative incidence, 1%), whereas incidence was highest in patients who were more than 55 years old at transplantation and who received a transplant more than 21 months after their initial lymphoma diagnosis (5-year cumulative incidence, 20%). CONCLUSION: Our study defines a subset of relapsed or refractory lymphoma patients who should be closely monitored for development of s-MDS/AML after high-dose chemotherapy and ASCT.