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Volume 47, Number 5, October 2014

An 11-year and 10-month-old girl with purpura and chest pain 


Pei-Hsuan Chen, Bor-Luen Chiang, Meng-Yao Lu, Yao-Hsu Yang


Received: February 29, 2012    Revised: April 30, 2012    Accepted: June 1, 2012   

 

Background and purpose: 

Mucosa-associated lymphoid tissue lymphoma (MALToma) is a type of B-cell lymphoma. Case reports of childhood thymic MALToma and its association with vasculitis are rarely found in the related literature. Herein, we present a report of an 11-year and 10-month-old girl who was initially diagnosed with cutaneous vasculitis characterized by nonthrombocytopenic palpable purpura, positive antinuclear antibody and anti-SSA (Ro) antibody. Eight months later, a thymic mediastinal mass was found. Surgical excision was performed and results of pathological analysis revealed an extranodal marginal zone CD20+ B-cell MALToma. Benign response to the chemotherapeutic regimen of Berlin–Frankfurt–Münster group NHL-BFM 90 R2 without relapse was noted in 2 years of follow-up. For the first time, our case demonstrated some clinical evidence of the association between vasculitis and childhood MALToma. 



 

Key words:

Anti-SSA (Ro) antibody, Mucosa-associated lymphoid tissue lymphoma (MALToma), Vasculitis