Splenic marginal zone lymphoma study

An SWG Grant-supported project initiated by the EHA Lymphona Group.  

Full project title  

Long-term outcomes in patients with SMZL treated in the rituximab era. 

Project lead  

Dr Ahmed Ludvigsen Al-Mashhadi 
Senior Registrar 
Aarhus University Hospital 
Denmark 

Project background and aims  

Splenic marginal zone lymphoma (SMZL) is a very rare, incurable, but indolent disease of the spleen with:  

  • Generally favorable outcomes  
  • High long-term survival rates  

Splenectomy is an effective treatment and resolves both symptoms and cytopenias related to splenomegaly in most patients.  

Rituximab, when used as a single agent in combination with chemotherapy, is also a viable treatment alternative.  

However, the long-term treatment outcomes are not well-established.  

There are no:  

  • Randomized clinical trials comparing these two treatment modalities  
  • Tools to aid clinicians in the decision between available treatment modalities  

In addition, randomized clinical trials are not likely to be conducted due to:  

  • The favorable outcomes in general—that is, the low number of events  
  • The rarity of the disease  

In our study, we wish to gather detailed medical records from more than a thousand patients across three continents and more than 25 centers in a retrospective, real-world setting.  

With this large dataset, we wish to:  

  • Describe the outcomes of SMZL  
  • Generate predictive risk scores for first-line treatment with rituximab-based therapies versus splenectomy, comparing survival outcomes  

 With the grant, we can finalize the study with the necessary statistical assistance and cover a significant portion of other study-related activities.  

Project updates  

You can find out more about the status of this project by reading the latest update from Dr Ahmed Ludvigsen Al-Mashhadi.