Georgia Malamut1, Bertrand Meresse2, Sophie Kaltenbach3, Coralie Derrieux4, Virginie Verkarre5, Elizabeth Macintyre4, Agnès Ruskone-Fourmestraux6, Bettina Fabiani7, Isabelle Radford-Weiss3, Nicole Brousse5, Olivier Hermine8, Nadine Cerf-Bensussan2, Christophe Cellier9. 1. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Gastroenterology Department, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France; Inserm U989, Paris, France; Institute Imagine, Paris, France. Electronic address: georgia.malamut@egp.aphp.fr. 2. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Inserm U989, Paris, France; Institute Imagine, Paris, France. 3. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Cytogenetic Department, Hôpital Necker Enfants Malades Assistance Publique des Hôpitaux de Paris, Paris, France. 4. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Biological Hematology Department, Hôpital Necker Enfants Malades Assistance Publique des Hôpitaux de Paris, Paris, France. 5. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Pathology Department, Hôpital Necker Enfants Malades Assistance Publique des Hôpitaux de Paris, Paris, France. 6. Gastroenterology Department, Hôpital Saint-Antoine Assistance Publique des Hôpitaux de Paris, Paris, France. 7. Pathology Department, Hôpital Saint-Antoine Assistance Publique des Hôpitaux de Paris, Paris, France. 8. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Institute Imagine, Paris, France; Hematology Department, Hôpital Necker Enfants Malades Assistance Publique des Hôpitaux de Paris, Paris, France. 9. Université Paris Descartes-Sorbonne Paris Centre, Paris, France; Gastroenterology Department, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France; Inserm U989, Paris, France; Institute Imagine, Paris, France.
Abstract
BACKGROUND & AIMS: Little is known about intestinal CD4+ T-cell lymphoma; this rare malignancy is misdiagnosed frequently. We evaluated diagnostic criteria and factors that might affect its development and outcome. METHODS: In a retrospective analysis, we analyzed medical records and intestinal specimens from 10 patients diagnosed with intestinal CD4+ T-cell lymphoma among 115 consecutive patients examined for severe enteropathy with villous atrophy. Samples were analyzed by histology, flow cytometry, and comparative genomic hybridization. RESULTS: Small-intestine epithelial and lamina propria tissues from patients who presented with chronic diarrhea and malnutrition had variable levels of infiltration of CD3+ CD4+ T cells. Flow cytometry showed a high frequency of CD4+ intraepithelial cells, which frequently expressed a specific Vβ chain. T-cell receptor β clonality was confirmed by DNA sequencing. Two patients had HLA and serology results compatible with celiac disease and autoimmune enteropathy, respectively. Two patients were found to have antibodies against human T-cell leukemia virus and 2 patients had signs of a recent infection with the herpes viruses. Comparative genomic hybridization analyses showed heterogeneous chromosomal abnormalities. Symptoms were reduced in patients treated with steroids (n = 5), but not in patients given purine analogues or chemotherapy. Antibodies against CD52 produced clinical and histologic responses in 2 of 2 patients, whereas severe adverse effects developed in 1 patient. At the latest follow-up evaluation, all patients were alive. CONCLUSIONS: There is much heterogeneity in the onset and genetic features of intestinal CD4+ T-cell lymphomas, despite their common presentation as indolent lymphoproliferations of the intestinal mucosa. Patients should be treated with steroids, and possibly antibodies against CD52 (for the most aggressive forms of this disorder).
BACKGROUND & AIMS: Little is known about intestinal CD4+ T-cell lymphoma; this rare malignancy is misdiagnosed frequently. We evaluated diagnostic criteria and factors that might affect its development and outcome. METHODS: In a retrospective analysis, we analyzed medical records and intestinal specimens from 10 patients diagnosed with intestinal CD4+ T-cell lymphoma among 115 consecutive patients examined for severe enteropathy with villous atrophy. Samples were analyzed by histology, flow cytometry, and comparative genomic hybridization. RESULTS: Small-intestine epithelial and lamina propria tissues from patients who presented with chronic diarrhea and malnutrition had variable levels of infiltration of CD3+ CD4+ T cells. Flow cytometry showed a high frequency of CD4+ intraepithelial cells, which frequently expressed a specific Vβ chain. T-cell receptor β clonality was confirmed by DNA sequencing. Two patients had HLA and serology results compatible with celiac disease and autoimmune enteropathy, respectively. Two patients were found to have antibodies against humanT-cell leukemia virus and 2 patients had signs of a recent infection with the herpes viruses. Comparative genomic hybridization analyses showed heterogeneous chromosomal abnormalities. Symptoms were reduced in patients treated with steroids (n = 5), but not in patients given purine analogues or chemotherapy. Antibodies against CD52 produced clinical and histologic responses in 2 of 2 patients, whereas severe adverse effects developed in 1 patient. At the latest follow-up evaluation, all patients were alive. CONCLUSIONS: There is much heterogeneity in the onset and genetic features of intestinal CD4+ T-cell lymphomas, despite their common presentation as indolent lymphoproliferations of the intestinal mucosa. Patients should be treated with steroids, and possibly antibodies against CD52 (for the most aggressive forms of this disorder).
Authors: Anamarija M Perry; Nathanael G Bailey; Michelle Bonnett; Elaine S Jaffe; Wing C Chan Journal: Int J Surg Pathol Date: 2018-07-09 Impact factor: 1.271
Authors: Craig R Soderquist; Nupam Patel; Vundavalli V Murty; Shane Betman; Nidhi Aggarwal; Ken H Young; Luc Xerri; Rebecca Leeman-Neill; Suzanne K Lewis; Peter H Green; Susan Hsiao; Mahesh M Mansukhani; Eric D Hsi; Laurence de Leval; Bachir Alobeid; Govind Bhagat Journal: Haematologica Date: 2019-09-26 Impact factor: 9.941