Abstract
Enteropathy-associated T-cell lymphoma (EATL) ultimately develops in 7–10% of patients with long-standing coeliac disease. In patients without a prior diagnosis of coeliac disease this is a very rare disorder, and the diagnosis in such cases is often difficult and delayed due to the non-specific nature of the symptoms and a very low index of clinical suspicion. Standard anti-lymphoma therapies have minimal utility in patients with EATL, and their prognosis is poor. An added difficulty is the high risk of intestinal perforation especially with the commencement of treatment due to the multifocal nature of bowel disease and poor underlying nutrition and tissue integrity. To illustrate these problems and provide an example of how these issues may be addressed, we report the case of a patient with EATL who was completely asymptomatic from unsuspected underlying coeliac disease and presented initially with back pain followed by bowel obstruction. He was treated with gut rest with total parenteral nutrition before commencing an intensive chemotherapy regimen [hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone)] and is currently well in ongoing complete remission 34 months later.
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References
Isaacson PG, O’Connor NT, Spencer J et al (1985) Malignant histiocytosis of the intestine: a T-cell lymphoma. Lancet 2:688–691
Lopez-Guillermo A, Cid J, Salar A et al (1998) Peripheral T-cell lymphomas: initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. classification. Ann Oncol 9:849–855
Ashton-Key M, Diss TC, Pan L et al (1997) Molecular analysis of T-cell clonality in ulcerative jejunitis and enteropathy-associated T-cell lymphoma. Am J Pathol 151:493–498
Carbonnel F, Grollet-Bioul L, Brouet JC et al (1998) Are complicated forms of celiac disease cryptic T-cell lymphomas? Blood 92:3879–3886
Catassi C, Fabiani E, Corrao G et al (2002) Risk of non-Hodgkin lymphoma in celiac disease. JAMA 287:1413–1419
Holmes GK, Prior P, Lane MR et al (1989) Malignancy in coeliac disease—effect of a gluten free diet. Gut 30:333–338
Collin P, Reunala T, Pukkala E et al (1994) Coeliac disease—associated disorders and survival. Gut 35:1215–1218
Green PH, Jabri B (2003) Coeliac disease. Lancet 362:383–391
Farrell RJ, Kelly CP (2002) Celiac sprue. N Engl J Med 346:180–188
Bevan S, Popat S, Braegger CP et al (1999) Contribution of the MHC region to the familial risk of coeliac disease. J Med Genet 36:687–690
Greco L, Romino R, Coto I et al (2002) The first large population based twin study of coeliac disease. Gut 50:624–628
Cellier C, Delabesse E, Helmer C et al (2000) Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet 356:203–208
Obermann EC, Diss TC, Hamoudi RA et al (2004) Loss of heterozygosity at chromosome 9p21 is a frequent finding in enteropathy-type T-cell lymphoma. J Pathol 202:252–262
Zettl A, Ott G, Makulik A et al (2002) Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma. Am J Pathol 161:1635–1645
de Bruin PC, Connolly CE, Oudejans JJ et al (1997) Enteropathy-associated T-cell lymphomas have a cytotoxic T-cell phenotype. Histopathology 31:313–317
Gupta S, Kim CH, Tsuruo T, Gollapudi S (1992) Preferential expression and activity of multidrug resistance gene 1 product (P-glycoprotein), a functionally active efflux pump, in human CD8+ T cells: a role in cytotoxic effector function. J Clin Immunol 12:451–458
Gale J, Simmonds PD, Mead GM et al (2000) Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol 18:795–803
Egan LJ, Walsh SV, Stevens FM et al (1995) Celiac-associated lymphoma. A single institution experience of 30 cases in the combination chemotherapy era. J Clin Gastroenterol 21:123–129
Daum S, Ullrich R, Heise W et al (2003) Intestinal non-Hodgkin’s lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin’s Lymphoma. J Clin Oncol 21:2740–2746
Kantarjian HM, O’Brien S, Smith TL et al (2000) Results of treatment with hyper-CVAD, a dose-intensive regimen, in adult acute lymphocytic leukemia. J Clin Oncol 18:547–561
Blystad AK, Enblad G, Kvaloy S et al (2001) High-dose therapy with autologous stem cell transplantation in patients with peripheral T cell lymphomas. Bone Marrow Transplant 27:711–716
Jantunen E, Wiklund T, Juvonen E et al (2004) Autologous stem cell transplantation in adult patients with peripheral T-cell lymphoma: a nation-wide survey. Bone Marrow Transplant 33:405–410
Rodriguez J, Munsell M, Yazji S et al (2001) Impact of high-dose chemotherapy on peripheral T-cell lymphomas. J Clin Oncol 19:3766–3770
Rodriguez J, Caballero MD, Gutierrez A et al (2003) High-dose chemotherapy and autologous stem cell transplantation in peripheral T-cell lymphoma: the GEL-TAMO experience. Ann Oncol 14:1768–1775
Jantunen E, Juvonen E, Wiklund T et al (2003) High-dose therapy supported by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma. Leuk Lymphoma 44:2163–2164
Buchmann I, Moog F, Schirrmeister H, Reske SN (2000) Positron emission tomography for detection and staging of malignant lymphoma. Recent Results Cancer Res 156:78–89
Mollee PN, Mills AK, Grimmett K et al (2003) The impact of the hyper-CVAD chemotherapy regimen on the ability to mobilize peripheral blood stem cells (PBSC) in patients with hematological malignancies. Blood 102:3572
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Hönemann, D., Prince, H.M., Hicks, R.J. et al. Enteropathy-associated T-cell lymphoma without a prior diagnosis of coeliac disease: diagnostic dilemmas and management options. Ann Hematol 84, 118–121 (2005). https://doi.org/10.1007/s00277-004-0953-9
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DOI: https://doi.org/10.1007/s00277-004-0953-9