Premium
A unique description of stage IV extranodal marginal zone lymphoma (EMZL) in an adolescent associated with gamma heavy chain disease
Author(s) -
Mittal Nupur,
Zhu Bing,
Gaitonde Sujata,
Lu Yang,
Schmidt Mary Lou
Publication year - 2015
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25419
Subject(s) - medicine , bendamustine , lymphoma , stage (stratigraphy) , disease , rituximab , chemotherapy , pathology , oncology , paleontology , biology
Extranodal Marginal zone lymphoma (EMZL) is a rare, usually localized disease in children. Advanced stage EMZL in adults is considered incurable, with prolonged remissions after chemotherapy. Gamma heavy chain disease (γHCD) is a rare disease of adults associated with lympho‐proliferative processes with no comparable reports in children. A case of stage‐IV EMZL with γHCD in an adolescent is discussed including treatment with Bendamustine plus Rituximab. The patient remains disease free 18 months from diagnosis. This case highlights necessity for careful diagnostic work‐up to identify indolent lymphomas in children which may respond to less toxic chemotherapy than used for common pediatric lymphomas. Pediatr Blood Cancer 2015;62:905–908. © 2015 Wiley Periodicals, Inc.