Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma

Gebhart, Johanna; Lechner, Klaus; Skrabs, Cathrin; Sliwa, Thamer; Müldür, Ercan; Ludwig, Hans; Nösslinger, Thomas; Vanura, Katrina; Stamatopoulos, Kostas; Simonitsch‐Klupp, Ingrid; Chott, Andreas; Quehenberger, Peter; Mitterbauer-Hohendanner, Gerlinde; Pabinger, Ingrid; Jäger, Ulrich; Geißler, Katharina

doi:10.1016/j.thromres.2014.08.021

Cited by 22 publications

(27 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Approximately 20% of patients show an autoimmune manifestation, 1 including autoimmune hemolytic anemia, immune thrombocytopenia, cold agglutinin disease, circulating anticoagulants, acquired von Willebrand disease, or angioedema as a result of acquired C1-esterase inhibitor deficiency. 2,3 Diagnosing SMZL and distinguishing it from similar indolent B-cell lymphoproliferative disorders can be challenging, especially if it relies on BM morphology and phenotype without the support of spleen histology. Although the majority of patients show an indolent course with a median survival of approximately 8 to 10 years, 4,5 the prognosis of SMZL is heterogeneous.…”

Section: Introductionmentioning

confidence: 99%

Splenic marginal zone lymphoma: from genetics to management

Arcaini

Rossi

Paulli

2016

Blood

141

132

View full text Add to dashboard Cite

Splenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy involving the spleen, bone marrow, and frequently the blood. SMZL lymphomagenesis involves antigen and/or superantigen stimulation and molecular deregulation of genes (NOTCH2 and KLF2) involved in the physiological differentiation of spleen marginal zone B cells. Diagnosis requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells coupled with the detection of intrasinusoidal infiltration by CD20+ cells in the bone marrow. Among B-cell tumors, deletion of 7q and NOTCH2 mutations are almost specific lesions of SMZL, thus representing promising diagnostic biomarkers of this lymphoma. Although the majority of SMZLs show an indolent course with a median survival of approximately 10 years, nearly 30% of patients experience a poor outcome. No randomized trials are reported for SMZL, and few prospective trials are available. A watch-and-wait approach is advisable for asymptomatic patients. Treatment options for symptomatic patients ranges from splenectomy to rituximab alone or combined with chemotherapy. In some geographic areas, a subset of patients with SMZL associates with hepatitis C virus infection, prompting virus eradication as an effective lymphoma treatment. It would be worthwhile to explore deregulated cellular programs of SMZL as therapeutic targets in the future; improved clinical and biological prognostication will be essential for identifying patients who may benefit from novel approaches.

show abstract

Section: Introductionmentioning

confidence: 99%

Splenic marginal zone lymphoma: from genetics to management

Arcaini

Rossi

Paulli

2016

Blood

141

132

View full text Add to dashboard Cite

show abstract

“…These studies showed that APLAs were seen in 41% and 26.6% of the NHL patients by Bairey et al and Genvresse et al . More specifically, a retrospective study investigating the prevalence of LA in MZL patients only showed elevated LA in 9 of 70 patients11 and 4 of those patients developed DVT. However, patients with asymptomatic or transient LA positivity may have been missed.…”

Section: Discussionmentioning

confidence: 99%

“…Treatment ranges from splenectomy, rituximab and chemotherapy with or without rituximab 18 19. In the restrospective study by Gebhart et al ,11 out of the nine cases with MZL, patients treated with rituximab – bendamustine achieved complete remission of LA whereas none of the patients treated with splenectomy alone had achieved complete remission. Although it is an underpowered study, we used BR immunochemotherapy for our patient with similar results.…”

Section: Discussionmentioning

confidence: 99%

“…The association of APLA and malignancy is a well-documented phenomenon 4. The prevalence of APLA was increased in cancer patients in one case-control study,5 three prospective cohort studies6–8 and three retrospective studies 9–11. Most of these studies also demonstrated an increase in thromboembolic events in this patient population, and several studies have shown that treatment of the underlying malignancy often leads to normalisation of APLA 12.…”

Section: Introductionmentioning

confidence: 95%

See 1 more Smart Citation

Marginal zone lymphoma-associated antiphospholipid antibodies successfully treated with bendamustine rituximab

Liu¹,

Markham

Mandernach

2019

BMJ Case Rep

View full text Add to dashboard Cite

A 46-year-old man presented with splenomegaly, abdominal adenopathy and profoundly elevated prothrombin time and partial thromboplastin time. He was diagnosed with marginal zone lymphoma (MZL) and small lymphocytic lymphoma, and the abnormal coagulation studies were secondary to the presence of a lupus anticoagulant. Optimal upfront therapy for MZL has not been established, and the incidence of antiphospholipid antibodies (APLA) in this patient population is rare. Following treatment with six cycles of bendamustine and rituximab with 2 years of rituximab maintenance, our patient remained in remission and his coagulation studies normalised. This report describes a case of successful treatment of APLA associated with MZL that resolved after treatment of the lymphoma.

show abstract

“…Some patients with suspected lymphoma usually have severe thrombocytopenia caused by immunemediated bone-marrow infiltration, concurrent infection, and other factors. [5][6][7] Patients and their relatives worry about the risks of intraoperative/ postoperative bleeding and refuse incisional biopsy, making the diagnosis and treatment difficult.…”

Section: Discussionmentioning

confidence: 99%

Thrombocytopenia Should not be Barrier for Lymph-node Biopsy

Gui-min¹

2016

UHOD

View full text Add to dashboard Cite

Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma

Cited by 22 publications

References 24 publications

Splenic marginal zone lymphoma: from genetics to management

Splenic marginal zone lymphoma: from genetics to management

Marginal zone lymphoma-associated antiphospholipid antibodies successfully treated with bendamustine rituximab

Thrombocytopenia Should not be Barrier for Lymph-node Biopsy

Contact Info

Product

Resources

About