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Thymic Mucosa-associated Lymphoid Tissue Lymphoma in an Adolescent Girl

Naithani, Rahul MD, DM; Ngan, Bo Y. MD, PhD, FRCP (C); Roifman, Chaim MD, FRCP (C); Crump, Michael MD; Baruchel, Sylvain MD, FRCP (C); Abla, Oussama MD

Journal of Pediatric Hematology/Oncology: October 2012 - Volume 34 - Issue 7 - p 552–555
doi: 10.1097/MPH.0b013e3182459f0a
Clinical and Laboratory Observations

Mucosa-associated lymphoid tissue (MALT) lymphoma is very rare in children. We report the first case of pediatric thymic MALT lymphoma in an adolescent Asian girl. She presented with chest pain, dyspnea, and low-grade fever. A large anterior mediastinal mass was biopsied that confirmed the diagnosis of MALT lymphoma with trisomy 18. The patient had secondary immunodeficiency with low NK cell count and high IgA and IgG levels. Because of the advanced stage and the presence of trisomy 18, she was treated with cyclophosphamide, vincristine, prednisone, and rituximab, followed by involved-field radiotherapy. She is currently undergoing maintenance therapy with rituximab and remains in complete remission at 13 months from diagnosis. Thymic MALT lymphoma should be suspected in any Asian child with a cystic thymic mass and autoimmune disease or hyperglobinemia. Because of the slow proliferation rate of this type of lymphoma, a long-term follow-up is needed.

*Department of Paediatrics, Division of Haematology/Oncology

Department of Paediatric Laboratory Medicine, Division of Pathology

Department of Paediatrics, Division of Immunology, The Hospital for Sick Children

§Medical Oncology, Princess Margaret Hospital, Toronto, ON, Canada

The authors declare no conflict of interest.

Reprints: Oussama Abla, MD, Department of Paediatrics, Division of Haematology/Oncology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada M5G 1X8 (e-mail: oussama.abla@sickkids.ca).

Received July 4, 2011

Accepted November 30, 2011

Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.