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

Autori
Dumic, Miroslav ; Radman, Ivo ; Krnic Nevena ; Nola, Marin ; Kusec, Rajko ; Begovic, Davor ; Labar, Boris ; Rados, Marko

Izvornik
Clinical lymphoma & myeloma (1557-9190) 7 (2007), 9; 590-593

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Nijmegen breakage syndrome ; diffuse large B-cell non-Hodgkin lymphoma ; immunodeficiency ; cancer ; rituximab

Sažetak
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/predniso ne) chemotherapy. He has been in complete remission for 3 years after finishing the treatment.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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