Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes

Montoro, Julia; Gallur, Laura; Merchán, Brayan; Molero, Antonieta; Roldán, Elisa; Martínez-Valle, Ferrán; Villacampa, Guillermo; Navarrete, Mayda; Ortega, Margarita; Castellví, Josep; Saumell, Sílvia; Bobillo, Sabela; Bosch, Francesc; Valcárcel, David

doi:10.1007/s00277-018-3302-0

Cited by 40 publications

(34 citation statements)

References 31 publications

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“…2,3 In addition, concomitant autoimmune diseases are common in MDS; in a recent study, 48% of patients with MDS had serological or clinical evidence of an autoimmune disease, which was found to be an independent marker for a worse prognosis. 4 IST with antithymocyte globulin (ATG), [5][6][7][8] cyclosporine, [9][10][11] or alemtuzumab, 12 as well as IST combinations of ATG with cyclosporine [13][14][15][16][17] or etanercept, 18 have all been studied in this context. Although some studies reported clinical activity with IST, others were not able to confirm a benefit of IST in unselected or older patients with MDS.…”

Section: Introductionmentioning

confidence: 99%

The use of immunosuppressive therapy in MDS: clinical outcomes and their predictors in a large international patient cohort

et al. 2018

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Most studies of immunosuppressive therapy (IST) in myelodysplastic syndromes (MDS) are limited by small numbers and their single-center nature, and report conflicting data regarding predictors for response to IST. We examined outcomes associated with IST and predictors of benefit in a large international cohort of patients with MDS. Data were collected from 15 centers in the United States and Europe. Responses, including red blood cell (RBC) transfusion independence (TI), were assessed based on the 2006 MDS International Working Group criteria, and overall survival (OS) was estimated by Kaplan-Meier methods. Logistic regression models estimated odds for response and TI, and Cox Proportional Hazard models estimated hazards ratios for OS. We identified 207 patients with MDS receiving IST, excluding steroid monotherapy. The most common IST regimen was anti-thymocyte globulin (ATG) plus prednisone (43%). Overall response rate (ORR) was 48.8%, including 11.2% (95% confidence interval [CI], 6.5%-18.4%) who achieved a complete remission and 30% (95% CI, 22.3%-39.5%) who achieved RBC TI. Median OS was 47.4 months (95% CI, 37-72.3 months) and was longer for patients who achieved a response or TI. Achievement of RBC TI was associated with a hypocellular bone marrow (cellularity < 20%); horse ATG plus cyclosporine was more effective than rabbit ATG or ATG without cyclosporine. Age, transfusion dependence, presence of paroxysmal nocturnal hemoglobinuria or large granular lymphocyte clones, and HLA DR15 positivity did not predict response to IST. IST leads to objective responses in nearly half the selected patients with the highest rate of RBC TI achieved in patients with hypocellular bone marrows.

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Section: Introductionmentioning

confidence: 99%

The use of immunosuppressive therapy in MDS: clinical outcomes and their predictors in a large international patient cohort

et al. 2018

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“…Fluctuations in platelet count may have suggested accompanied autoimmune mechanism, immune thrombocytopenia. 7 …”

Section: Case Reportmentioning

confidence: 99%

Successful Long-Term Treatment with Azacitidine in Patient with Chronic Myelomonocytic Leukemia

et al. 2020

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CMML is clinically diverse malignant clonal hematopoietic stem cell disorder characterized by persistent peripheral monocytosis, bone marrow dysplasia and with features of both myelodysplastic and myeloproliferative neoplasms (1). Generally, long term prognosis is poor with a median overall survival of 30 months and a relatively high risk of leukemic transformation, up to 30 % (2). Hypomethylating agents belong to a class of drugs which inhibit DNA methyltransferase and consequently reduce DNA methylation. Utilization of HMA in CMML has been reported with seemingly unsatisfying results with overall response rates between 30 and 60% and median overall survival between 12 and 37 months (3,4). Previously only a few predictive factors (increased bone marrow blast count and proliferative features) were found to be associated with shorter survival (5). Surprisingly only age, but none of the other disease characteristics, was a prediction for a response(6). In this case report, we describe an effective long term use of azacitidine in a patient with CMML and tried to find factors, which may contribute to long term azacitidine efficacy.

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“…Autoimmune hemolytic anemia has been reported in association with MDS [57][58][59]. Association of systemic lupus erythematosus and MDS has been reported [60][61][62].…”

Section: Miscellaneousmentioning

confidence: 99%

Myelodysplastic Syndrome and Autoimmune Disorders: Causal Relationship or Coincidence?

Newman¹,

Akhtari²,

Heidarian³

2019

Recent Developments in Myelodysplastic Syndromes

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Myelodysplastic syndromes are heterogeneous group of clonal hematologic malignancies characterized by peripheral blood cytopenias secondary to the ineffective hematopoiesis. ADs are frequently reported in MDS, the incidence ranging from 10 to 30%, and particularly ADs are more frequently seen at CMML. ADs may prone patient to MDS, especially when immune suppressors such as azathioprine are used for the underlying AD. Both innate and adaptive immune systems, and different cytokines including interleukins, TNF-α, and C-X-C motif chemokine 10 (CXCL10) contribute in immune dysregulation of MDS. Vasculitis, seronegative rheumatoid arthritis, SLE, Behçet's disease, RP, and AIHA are just some of the ADs occurring concomitantly with MDS. Although hematopoietic growth factors are recommended by the American Society of Clinical Oncology (ASCO), it has been recognized from several case reports that treatment of the underlying MDS may resolve the associated autoimmune disorders. The heterogeneity and complexity of pathology, clinical manifestations, response to therapy, and prognosis of MDS and its immune dysregulation make the prognosis of MDS with autoimmune diseases a matter of debate. Better understanding of the immune dysregulation of MDS in the molecular level may help to design prospective, double blind clinical trials to find the best treatment options for autoimmune disorders associated with MDS.

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Autoimmune disorders are common in myelodysplastic syndrome patients and confer an adverse impact on outcomes

Cited by 40 publications

References 31 publications

The use of immunosuppressive therapy in MDS: clinical outcomes and their predictors in a large international patient cohort

The use of immunosuppressive therapy in MDS: clinical outcomes and their predictors in a large international patient cohort

Successful Long-Term Treatment with Azacitidine in Patient with Chronic Myelomonocytic Leukemia

Myelodysplastic Syndrome and Autoimmune Disorders: Causal Relationship or Coincidence?

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