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Editorials
References
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3. Li BE, Ernst P. Two decades of leukemia oncoprotein epistasis: the MLL1 paradigm for epigenetic deregulation in leukemia. Exp Hematol. 2014;42(12):995-1012.
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T-cell and NK-cell neoplasms of the gastrointestinal tract – recurrent themes, but clinical and biological distinctions exist
Elaine S. Jaffe
Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
E-mail: ELAINE S. JAFFE - ejaffe@mail.nih.gov doi:10.3324/haematol.2020.252924
The history of intestinal T-cell lymphomas begins with the early work of Peter Isaacson and Dennis Wright who described cases of “malignant histio- cytosis” of the intestine that they linked to malabsorption and ulcerative jejunitis.1 Subsequent work showed that “malignant histiocytosis of the intestine” was a form of T- cell lymphoma, later named enteropathy-associated T- cell lymphoma (EATL).2 Since then, we have come to understand the distinction between EATL, closely linked to celiac disease, and monomorphic epitheliotropic T-cell lymphoma (MEITL), formerly EATL type II (Figure 1).3 The work of Isaacson and Wright shaped the modern classification of both T-cell and B-cell intestinal lym- phomas, giving us not only EATL, but also mucosa-asso- ciated lymphoid tissue (MALT) lymphoma. Sadly Dennis Wright passed away on April 08, 2020 at the age of 88.
Most cases of intestinal T-cell lymphoma were highly aggressive, but in the 1990s there was a series of reports of low-grade intestinal T-cell neoplasms, some of which mimicked lymphomatous polyposis.4-8 The nature of this rare form of T-cell lymphoma was better defined in sub-
sequent reports,9,10 and incorporated into the Revised 4th
Edition of the World Health Organization (WHO) classi-
3
fication as a provisional entity under the term indolent T-
cell lymphoproliferative disorder of the gastrointestinal tract (ITLPD-GIT) (Figure 1). Most patients had a chronic, relapsing clinical course, although in both of the above series late instances of large-cell transformation were described.10,11
In the current issue of Haematologica, Soderquist et al. expand our knowledge regarding the immunophenotypic spectrum of ITLPD-GIT and provide new insights into its molecular pathogenesis.12 As with prior reports, all cases were derived from αβ T cells with an equal proportion of cases expressing either CD4 or CD8. One case each had either a double-positive or double-negative phenotype. The authors also examined the expression of T-bet (TBX21) and GATA3, but any conclusions regarding the functional or clinical significance of these markers, which have been examined more extensively in nodal peripheral T-cell lymphomas,13 remain premature.
This study confirms the importance of alterations in
haematologica | 2020; 105(7)