Successful treatment of diffuse large B-cell non-hodgkin lymphoma with modified CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy and rituximab in a patient with Nijmegen syndrome

Clinical Lymphoma & Myeloma
Miroslav DumicMarko Rados

Abstract

A 17-year-old Croatian boy with Nijmegen breakage syndrome (NBS) who developed diffuse large B-cell non-Hodgkin lymphoma is presented. The majority of the patients with this rare autosomal recessive disease are of Slavic origin and, in most of them, the disease is caused by NBS1 mutation 657del5, as was found in our patient. Nijmegen breakage syndrome is characterized by microcephaly, growth retardation, abnormal facial appearance, spontaneous chromosomal rearrangements, immunodeficiency, and a high predisposition to cancer development, predominantly lymphoma. Because of increased sensitivity to radiation therapy and chemotherapy, the treatment of malignancies in patients with NBS can be difficult. To our knowledge, our patient is the first with NBS reported in the literature who was successfully treated for diffuse large B-cell lymphoma with the anti-CD20 monoclonal antibody rituximab in addition to a modified dose of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy. He has been in complete remission for 3 years after finishing the treatment.

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Citations

Mar 1, 2012·Orphanet Journal of Rare Diseases·Krystyna H ChrzanowskaMartin Digweed
May 1, 2010·Cellular and Molecular Life Sciences : CMLS·Laurence MeyerAyikoe Guy Mensah-Nyagan
Jan 31, 2016·European Journal of Medical Genetics·Agata PastorczakUNKNOWN International Berlin-Frankfurt-Munster (I-BFM) ALL host genetic variation working group
Oct 22, 2008·Pediatric Blood & Cancer·Bozenna Dembowska-BaginskaKrystyna H Chrzanowska
Mar 10, 2005·Leukemia Research·Jun MatsuiJunichiro Fujimoto
Jun 3, 2017·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Michael F WalshSharon A Savage

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