Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma

Thromb Res. 2014 Nov;134(5):980-4. doi: 10.1016/j.thromres.2014.08.021. Epub 2014 Aug 29.


Introduction: Splenic marginal zone lymphoma (SMZL) is a rare low-malignant Non-Hodgkin lymphoma (NHL), in which immune mediated paraneoplastic phenomena such as autoimmune hemolytic anemia (AIHA), autoimmune thrombocytopenia (ITP) and C1 esterase inhibitor deficiency are relatively common.

Materials and methods: We performed a multicenter retrospective study in 70 patients on the prevalence and clinical features of antiphospholipid antibodies (aPLA) in SMZL.

Results and conclusions: Nine patients (13%) had the diagnosis of a lupus anticoagulant (LA). The occurrence of venous thromboembolic events was significantly higher in LA positive patients compared to LA negative patients (4/9 [44%] vs 5/61 [8%], p = 0.002), especially within 12 months after splenectomy (3/6 [50%] vs 2/28 [7%], p = 0.007). None of the patients with LA had a persistent complete remission of LA after splenectomy, but complete remission of LA was achieved in 2/2 patients after rituximab-bendamustine immuno-chemotherapy. In conclusion, our data show a relatively high prevalence of aPLA in SMZL and an increased risk of postsplenectomy thrombosis in these patients. The fact that rituximab-bendamustine was effective for eradicating LA may be considered as an argument for using immuno-chemotherapy as first line therapy in SMZL patients with LA.

Keywords: Antiphospholipid antibodies; Lupus anticoagulant; SMZL; Splenectomy; Splenic marginal zone lymphoma; Thrombosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use
  • Antineoplastic Agents / therapeutic use
  • Female
  • Humans
  • Immunotherapy
  • Lupus Coagulation Inhibitor / analysis*
  • Lymphoma, B-Cell, Marginal Zone / complications*
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Male
  • Middle Aged
  • Retrospective Studies
  • Rituximab
  • Spleen / pathology
  • Splenectomy
  • Splenic Neoplasms / complications*
  • Splenic Neoplasms / pathology
  • Splenic Neoplasms / therapy
  • Thrombosis / complications*
  • Treatment Outcome


  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Agents
  • Lupus Coagulation Inhibitor
  • Rituximab