From the Guidelines
The diagnosis of splenic marginal zone lymphoma (SMZL) can be established without a splenectomy specimen in most instances, through a combination of peripheral blood/bone marrow aspirate morphology and flow cytometry, as well as bone marrow biopsy histology and IHC, reviewed by expert haematopathologists/haematologists, as stated in the ESMO clinical practice guidelines for diagnosis, treatment and follow-up 1.
Key Considerations
- The accuracy and reproducibility of this approach have not yet been fully investigated, and in a minority of cases, the definitive diagnosis may not be possible without splenectomy.
- Neoplastic lymphocytes in SMZL often display villi; however, these are not seen in all cases and not every lymphoma displaying villi corresponds to SMZL.
- The role of PET is uncertain, and SMZL is usually staged by CT, with abdominal sonography giving additional information for the detection of splenic focal lesions 1.
Diagnostic Approach
- The examination of peripheral blood films and immunophenotyping allows for a diagnosis to be established in most cases, with the neoplastic cells being twice the size of a lymphocyte and having a round or kidney-shaped nucleus with loose chromatin and abundant pale cytoplasm with projections.
- Flow cytometry studies using anti-B-cell monoclonal antibodies such as CD19, CD20 or CD22, together with a panel of antibodies such as CD11c, CD25, CD103 and CD123, can confirm the diagnosis and differentiate SMZL from other B-cell leukaemias and lymphomas with circulating villous cells.
Treatment Considerations
- Treatment is only indicated for symptomatic disease and may include splenectomy, rituximab monotherapy, or rituximab-based chemoimmunotherapy like R-bendamustine, as stated in the ESMO clinical practice guidelines for diagnosis, treatment and follow-up 1.
- The decision to treat should be individualised according to the specific histological subtype and the corresponding current therapeutic guidelines, with a personalised therapeutic approach driven by the haematologist/oncologist, as recommended by the EULAR guidelines for the management of Sjögren's syndrome with topical and systemic therapies 1.
From the Research
Splenic Marginal Zone
The splenic marginal zone is a critical area in the spleen, playing a vital role in the immune system.
- The spleen is a frequently injured organ, with over 40,000 adult splenic injuries occurring yearly 2.
- In cases of blunt injury, indications for emergent splenectomy include hemodynamic instability and peritonitis 2.
- Nonoperative management has been successful for all grades of blunt injury (80-95%), but careful patient selection is crucial 2.
Diagnosis and Management
Diagnosis and management of splenic injuries involve various techniques, including:
- Computed tomography (CT) scan with intravenous contrast to classify the grade of injury and identify active bleeding and its stigmata 2.
- Angioembolization as an important tool for splenic salvage when an actively bleeding vessel is observed on CT scan 2.
- Close monitoring, including interval CT scans for high-grade injuries, for patients selected for nonoperative management 2.
Splenic Preservation
Splenic preservation is a key consideration in the management of splenic injuries, with various techniques available:
- Splenorrhaphy, or surgical repair of the spleen, has been reported to have successful results, with excellent healing capabilities 3.
- Splenic preserving techniques may be used to maintain function when surgery is unavoidable 4.
- The importance of preserving the spleen is highlighted by the increased risk of overwhelming postsplenectomy sepsis in splenectomized patients 3.