title: Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
abstract: BACKGROUND
  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.
  
  
  METHODS
  We performed a multicenter retrospective study in 70 patients on the prevalence and clinical features of antiphospholipid antibodies (aPLA) in SMZL.
  
  
  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.
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: J
authors.initials: J
authors.lastname: Gebhart
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Lechner
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: C
authors.initials: C
authors.lastname: Skrabs
authors.affiliation: 5thDepartment of Medicine, Krankenhaus Hietzing, Vienna, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Sliwa
authors.affiliation: Department of Medicine I, Wilhelminenspital Vienna, Vienna, Austria.
authors.forename: E
authors.initials: E
authors.lastname: Müldür
authors.affiliation: Department of Medicine I, Wilhelminenspital Vienna, Vienna, Austria.
authors.forename: H
authors.initials: H
authors.lastname: Ludwig
authors.affiliation: Third Medical Department for Hematology and Oncology and the Ludwig Boltzmann Institute for Leukemia Research and Hematology, Hanusch Hospital, Vienna, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Nösslinger
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Vanura
authors.affiliation: Hematology Department, G. Papanicolaou Hospital, Thessaloniki, Greece.
authors.forename: K
authors.initials: K
authors.lastname: Stamatopoulos
authors.affiliation: Institute of Pathology, Medical University Vienna, Vienna, Austria.
authors.forename: I
authors.initials: I
authors.lastname: Simonitsch-Klupp
authors.affiliation: Institute of Pathology, Wilhelminenspital Vienna, Vienna, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Chott
authors.affiliation: Institute of Laboratory Medicine, Medical University Vienna, Vienna, Austria.
authors.forename: P
authors.initials: P
authors.lastname: Quehenberger
authors.affiliation: Institute of Laboratory Medicine, Medical University Vienna, Vienna, Austria.
authors.forename: G
authors.initials: G
authors.lastname: Mitterbauer-Hohendanner
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: I
authors.initials: I
authors.lastname: Pabinger
authors.affiliation: Department of Medicine I, Clinical Division of Hematology and Hemostaseology and the Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: U
authors.initials: U
authors.lastname: Jäger
authors.affiliation: 5thDepartment of Medicine, Krankenhaus Hietzing, Vienna, Austria. Electronic address: klaus.geissler@wienkav.at.
authors.forename: K
authors.initials: K
authors.lastname: Geissler
mesh_terms: D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007167:Immunotherapy; D016682:Lupus Coagulation Inhibitor / Q000032:analysis*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab; D013154:Spleen / Q000473:pathology; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D013927:Thrombosis / Q000150:complications*; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D016682:Lupus Coagulation Inhibitor; D000069283:Rituximab
keywords: Antiphospholipid antibodies; Lupus anticoagulant; SMZL; Splenectomy; Splenic marginal zone lymphoma; Thrombosis