Splenic marginal zone lymphoma (SMZL)

  • Method:
  • Anticoagulant:
  • Recommendation:
  • Method:
    Cytomorphology
  • Anticoagulant:
    EDTA
  • Recommendation:
    obligatory
  • Method:
    Immunophenotyping
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory
  • Method:
    Chromosome analysis
  • Anticoagulant:
    Heparin
  • Recommendation:
    facultative
  • Method:
    FISH
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    facultative
  • Method:
    Molecular genetics
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    facultative

The splenic marginal zone lymphoma (SMZL) belongs to the group of marginal zone lymphomas and is a rare mature B-cell neoplasm with indolent clinical course. The annual incidence is 0.13/100,000 persons (Liu et al. 2013). The disease occurs median in the 6th decade of life and manifests itself in the spleen with frequent involvement of bone marrow and peripheral blood. The splenic marginal zone lymphoma is formed by B cells from the marginal zone of the white splenic pulp (Swerdlow et al. 2017).

The aetiology of the splenic marginal zone lymphoma is not clear, but immunological stimulation seems to play a role besides acquired pathogenic mutations in oncogenes and tumor suppressor genes. Epidemiological studies also point to a connection to viral infections with human herpes virus 8 and hepatitis C virus (HCV) (Benavente et al. 2011, Hermine et al. 2002).

Classification of splenic marginal zone lymphoma

The group of marginal zone lymphomas accounts for up to 9% of non-Hodgkin's lymphomas (The non-Hodgkin's lymphoma classification project 1997). Based on the localization of the primary tumor, the WHO classification (2017) distinguishes three entities:

  • splenic marginal zone lymphoma (SMZL)

  • the extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

  • nodal marginal zone lymphoma (NMZL) and the subtype of paediatric nodal marginal zone lymphoma

All three entities have the indolent course and the associated favorable prognosis in common.

SMZL - Diagnostics

SMZL: Prognosis

The splenic marginal zone lymphoma is an indolent disease. The prognosis is usually very good and the median survival is 10 years. However, in 5-10% of the patients a transformation into a large B-cell lymphoma occurs, especially into a diffuse large B-cell lymphoma (Xing et al. 2015). The prognosis for patients with transformed SMZL is adverse (Florindez et al. 2019).

In splenic marginal zone lymphoma, a prognostic score has not yet been established. The splenic marginal zone lymphoma study group is striving for risk stratification, and its prognostic index takes the following parameters into account: haemoglobin, platelet count, lactate dehydrogenase, extrahilar lymphadenopathy (Kalpadakis et al. 2014) The Intergruppo Italiano Linformi Index (IIL) also includes the clinical parameters of haemoglobin and lactate dehydrogenase, and the IIL also includes serum albumin (Arcaini et al. 2006). Mutations of the TP53 gene are associated with a worse prognosis and patients with mutations of NOTCH2 and KLF2 have been observed to require earlier treatment (Parry et al. 2015).

Splenic marginal zone lymphoma - Recommendation

According to the current guideline of the "European Society for Medical Oncology" (ESMO) on marginal zone lymphoma, the diagnosis of splenic marginal zone lymphoma requires besides the collection of clinical and laboratory parameters a cytomorphological and immunophenotypical analysis of peripheral blood and bone marrow aspirate as well as a histological and immunohistochemical examination of the bone marrow. In a small percentage of cases, splenectomy is necessary for the diagnosis or exclusion of a splenic diffuse small cell B-cell lymphoma of the red pulp (SDRPL) (Zucca et al. 2020).

Therapy is usually initiated if progressive or symptomatic splenomegaly is present and/or any progressive cytopenia is detectable (haemoglobin <10 g/dl, platelets <80,000/µl, neutrophils <1000/µl) (Zucca et al. 2020). The assessment of the therapy response requires, like the diagnosis, the interaction of methods of cytomorphology, immunophenotyping as well as histology and immunohistochemistry. Thus, in addition to the clinical parameter of spleen size, hematological recovery, the proportion of circulating clonal cells in the blood and the extent of bone marrow infiltration are assessed (Zucca et al. 2020).

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