Document Detail

Pediatric nodal marginal zone lymphoma may develop in the adult population.
MedLine Citation:
PMID:  19863176     Owner:  NLM     Status:  MEDLINE    
Pediatric nodal marginal zone lymphoma (NMZL) is described as a separate variant of NMZL in the most recent WHO classification of tumors of hematologic and lymphoid tissues. It has distinctive morphology and clinical presentation and stands out as an indolent disease with remarkably better overall prognosis compared to classic NMZL. Here we report two adult patients with NMZL with clinical and morphologic features consistent with pediatric NMZL (pNMZL) and review available literature describing the clinical and histologic presentation of pNMZL. Two men, ages 44 and 18 years, each presented with localized cervical lymphadenopathy, both demonstrated florid proliferation of the marginal zone and disruption of reactive germinal centers, progressive transformation of germinal centers-like morphologic features typical for pNMZL and clonal disease with immunophenotype consistent with NMZL. This is the first report of pNMZL in a middle-aged person. Distinct histologic features and characteristic benign clinical course will help to distinguish this rare variant from other NMZL in the adults. Clinically, recognition is important to understand the true incidence of this rare form in the adult population and to avoid unnecessary overtreatment of this indolent form.
Elena Gitelson; Tahseen Al-Saleem; Valentin Robu; Michael M Millenson; Mitchell R Smith
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Leukemia & lymphoma     Volume:  51     ISSN:  1029-2403     ISO Abbreviation:  Leuk. Lymphoma     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2010-01-08     Completed Date:  2010-04-02     Revised Date:  2013-04-12    
Medline Journal Info:
Nlm Unique ID:  9007422     Medline TA:  Leuk Lymphoma     Country:  England    
Other Details:
Languages:  eng     Pagination:  89-94     Citation Subset:  IM    
Department of Medical Oncology, Fox Chase Cancer Center, PA 19111, USA.
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MeSH Terms
Antibodies, Monoclonal / therapeutic use
Antibodies, Monoclonal, Murine-Derived
Antineoplastic Agents / therapeutic use
Flow Cytometry / methods
Immunotherapy / methods
Lymphatic Diseases / diagnosis,  pathology
Lymphoma, B-Cell, Marginal Zone / diagnosis*,  immunology,  surgery,  therapy
Medical Oncology / methods*
Remission Induction
Treatment Outcome
Grant Support
R01 CA071552-03/CA/NCI NIH HHS
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Antibodies, Monoclonal, Murine-Derived; 0/Antineoplastic Agents; 0/rituximab

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