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

Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with splenic marginal zone lymphoma (SMZL). We have summarized the most important info on classification and diagnostic methods at MLL. In addition, we provide further links and literature on prognosis and therapy in SMZL, so that you can inform yourself in more detail.

Splenic marginal zone lymphoma: Classification

Splenic marginal zone lymphoma (SMZL) belongs to the group of splenic B-cell lymphomas and is a rare mature B-cell neoplasm with an indolent clinical course. The disease occurs in the median 7th decade of life and manifests in the spleen with frequent involvement of bone marrow and peripheral blood. The spleen usually shows involvement of both the white and red pulp, with the white pulp typically dilated (WHO 2022). The marginal zone lymphoma group accounts for up to 9% among non-Hodgkin's lymphomas (The non-Hodgkin's lymphoma classification project 1997). The WHO distinguishes the following entities within splenic B-cell lymphomas and leukemias (WHO 2022):

  • Hairy cell leukemia (HZL)
  • Splenic marginal zone lymphoma (SMZL)
  • Splenic diffuse small B-cell lymphoma of the red pulp (SDRPL)
  • Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN)

Splenic marginal zone lymphoma: Diagnostic methods and their relevance

Splenic marginal zone lymphoma: Prognosis

Splenic marginal zone lymphoma is an indolent disease with usually good prognosis and a median survival of more than 10 years (WHO 2022). However, transformation to large B-cell lymphoma, especially diffuse large B-cell lymphoma, occurs in 5-10% of patients (Xing et al. 2015). The prognosis for patients with transformed SMZL is unfavorable (Florindez et al. 2020, WHO 2022). No prognostic score has yet been firmly established. Efforts at risk stratification exist on the part of various working groups (Arcaini et al. 2006, Montalbán et al. 2012, Kalpadakis et al. 2014, Montalban et al. 2014). Mutations of the TP53 gene and mutations of NOTCH2 and KLF2 are associated with poorer prognosis and earlier need for treatment, respectively (Parry et al. 2015, WHO 2022).

Splenic marginal zone lymphoma: Recommendation and therapy

According to the current guideline of the European Society for Medical Oncology (ESMO) on marginal zone lymphoma, the diagnosis and assessment of response to therapy of splenic marginal zone lymphoma require, in addition to the collection of clinical and laboratory parameters, cytomorphologic and immunophenotypic analysis of peripheral blood and bone marrow aspirate as well as histologic and immunohistochemical examination of the bone marrow. In a small proportion of cases, splenectomy is necessary to establish the diagnosis or to exclude splenic diffuse small B-cell lymphoma of the red pulp (SDRPL) (Zucca et al. 2020).

Generally, therapy is initiated for progressive/symptomatic splenomegaly and/or any progressive cytopenia that is detectable (Zucca et al. 2020).

Status: December 2023

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