University of Nebraska Medical Center, Omaha, NE 68198, USA
* Email: eryan{at}unmc.edu
The next, and largely unexplored, frontier in lymphoma management is the problem of T-cell lymphomas. In western countries, T-cell lymphomas represent <10% of all non-Hodgkin's lymphomas or approximately 5000 new cases per year in the USA. However, T-cell lymphomas are not uniform and an increasing number of subtypes are being recognized. Lymphoblastic lymphomas are tumors of precursor T-cells and frequently present as leukemia or with a mediastinal mass in young men. Lymphomas of more mature T-cells are referred to as peripheral T-cell lymphomas. This name is sometimes confusing as it does not refer to sites of involvement but rather to more matureor post-thymicT-cell lymphomas. The current World Health Organization (WHO) classification of the peripheral T-cell lymphomas is listed in Table 1 [1]. Most are aggressive neoplasms with only mycosis fungoides and cutaneous anaplastic large-cell lymphoma frequently following an indolent course. Several of these disorders present as distinctive clinicalpathologic syndromes (e.g. enteropathy type in patients with celiac disease), while others are only distinguished from B-cell lymphoma by immunophenotyping. Many of these lymphomas are quite rare, but the frequency of the more common peripheral T-cell lymphomas, as found in a large international study, is shown in Table 2 [2
4
].
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In this issue of the Annals of Oncology, the lymphoma team from Vancouver present their analysis of peripheral T-cell lymphomas seen at the British Columbia Cancer Agency [20]. We know that peripheral T-cell lymphomas frequently vary in incidence geographically [21
]. In this regard the relatively large number of extra-nodal NK/T-cell nasal lymphomas seen in Vancouver might reflect the large immigrant population from China and Southeast Asia. They found a poorer outcome for patients with the anaplastic large-cell type than has been found in some studies. This might relate to exclusion of the null-cell subgroup, which is often ALK positive. In addition, they found a lower frequency of angioimmunoblastic T-cell lymphoma than has been seen in previous studies. It would be interesting to know if that lymphoma is truly less common in British Columbia and, if so, why. Their paper confirms many previous observations about this difficult group of lymphomas and is an important contribution to stimulate further studies.
There is great room for improvement in our management of patients with the peripheral T-cell lymphomas. Studies of gene and protein expression need to be done to refine our ability to classify T-cell lymphomas, to find subgroups that are especially likely to benefit from our current treatments, and to identify possible targets for new therapies. We are desperately in need of clinical trials that focus specifically on T-cell lymphomas. However, their relative rarity will make it difficult for any individual institution to carry out these trials. Cooperative studies, perhaps on an international basis, might be necessary to improve our understanding of the biology and the treatment of the peripheral T-cell lymphomas. Our patients need us to undertake these efforts in an expeditious manner.
References
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