Celiac.com 03/16/2010 - Enteropathy associated T-cell lymphoma (EATL)  is a rare type of peripheral T-cell lymphoma that is commonly associated with celiac disease.  A group at The Newcastle  Lymphoma Group in the  United Kingdom, evaluated data from newly diagnosed patients in Northern England and Scotland between 1994 and 1998, in search of increased overall survival (OS) rates and progression free survival (PFS) rates for EATL patients.

Celiac disease (CD) is the most common food intolerance disorder affecting Western civilization today. While most celiacs show an improvement in their health after  initiating a gluten free diet,  2-5%  of patients do not improve, and are thus considered to have refractory celiac disease (RCD).

RCD is further classified into two categories, Type 1 with intraepithelial lymphocytes of normal phenotype, or as type 2 with clonal expansion of intraepithelial lymphocytes with an aberrant phenotype. Type 2 patients are expected to have a five year overall survival rate (OS) of 50%-58%, and most Type 2 RCD  patients die from EATL.

EATL generally affects older patients in their 60's or 70's, with a history of CD or RCD, and is most frequently presented in the form of malabsorption along with abdominal pain. However, EATL is not exclusive to patients with CD or RCD and has also been found in patients without a history of either. Standard treatments until now have included surgical resection, with or without anthracycline-based chemotherapy, or high-dose chemotherapy with autologous stem cell transplant (ASCT). Results of these treatments have been dismal, with the patient typically dying from disease related complications.

Using a population-based setting, 26 EATL patients that qualified for intensive treatment were given the new aggressive treatment of, ifosfamide, vincristine, etoposide / methotrexate (IVE/MTX) &  ASCT, and their results were compared to that of the historical group. Statistically there was no difference between the groups; all groups had similar age, sex and features at initial evaluation. For all patients treated with the historical cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) therapy, the average PFS rate was approximately three months, and the average OS rate was about seven months. However,  the IVE/MTX - ASCT group showed a significantly higher five year PFS and OS compared to patients treated with the  historical CHOP therapy. Additionally, patients treated with IVE/MTX - ASCT showed improvement in their remission rates, and had profound reduction of death rates compared to the group treated with the historical CHOP chemotherapy. Of the patients that were solely treated with surgery, none survived.

While EATL has a somber outcome for most patients treated with conventional CHOP treatments, data collected from these tests reveal that the regime IVE/MTX – ASCT shows exceptional promise as a new treatment. It is recommended that EATL patients enter themselves into national studies like this one, to expand research data and to help explore potentially effective EATL treatment options.

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