Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review

Wesner, N.; Drevon, Louis; Guédon, A.; Fraison, Jean Baptiste; Terrier, Benjamin; Trad, S.; Kahn, Jean‐Emmanuel; Aouba, Achille; Gillard, J.; Ponsoye, Matthieu; Hanslik, Thomas; Gourguechon, C.; Liozon, É.; Laribi, Kamel; Rossignol, Julien; Hermine, Olivier; Seksik, Philippe; Adès, Lionel; Carrat, Fabrice; Fenaux, Pierre; Mékinian, A.; Fain, Olivier

doi:10.1080/10428194.2018.1542152

Cited by 28 publications

(19 citation statements)

References 25 publications

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“…MDS-vasculitis patients are generally younger with a prevalence of male sex, as compared to MDS without vasculitis [68]. No correlations with specific MDS subtypes have been reported [69], except for Behçet's-like syndrome and trisomy 8 [70]. Regarding vasculitis subtypes, the most common are polyarteritis nodosa and giant-cell arteritis.…”

Section: Aid In Mdsmentioning

confidence: 98%

“…The latter displays a milder clinical course compared to the idiopathic form (less frequent headaches, jaw claudication and optic neuropathy). Moreover, MDS-associated Behçet's-like syndrome has a delayed onset, greater gastrointestinal and oral involvement and reduced ocular manifestations than the idiopathic form [70,71]. Other frequent associations are relapsing polychondritis and SLE, whilst SS, RA, polymyalgia rheumatica, uveitis, pulmonary proteinosis, thyroiditis, and myositis are sporadically reported [50,68,94].…”

Section: Aid In Mdsmentioning

confidence: 99%

“…Some large retrospective studies suggest a reduced overall survival for MDS patients with AID, especially for cryoglobulinemic vasculitis, due to higher infectious rate [50]. Conversely, other studies reported no impact on survival, even if increased cardiovascular comorbidity has been reported in MDS with AID [69,70,73]. A large multicenter study involving more than 1,400 MDS patients with autoimmunity suggests that AID-MDS may be associated with rather better overall and leukemia-free survival [74,75].…”

Section: Aid In Mdsmentioning

confidence: 99%

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Autoimmune Complications in Hematologic Neoplasms

Barcellini

Giannotta

Fattizzo

2021

Cancers

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Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. The latter include chronic lymphocytic leukemia (CLL), lymphomas, multiple myeloma, myelodysplastic syndromes (MDS), chronic myelomonocytic leukemia (CMML), myeloproliferative neoplasms, and acute leukemias. On the whole, AICy are observed in up to 10% of CLL and with higher frequencies in certain subtypes of non-Hodgkin lymphoma, whilst they occur in less than 1% of low-risk MDS and CMML. AID are described in up to 30% of myeloid and lymphoid patients, including immune-mediated hemostatic disorders (acquired hemophilia, thrombotic thrombocytopenic purpura, and anti-phospholipid syndrome) that may be severe and fatal. Additionally, AICy/AID are found in about 10% of patients receiving hematopoietic stem cell transplant or treatment with new checkpoint inhibitors. Besides the diagnostic difficulties, these AICy/AID may complicate the clinical management of already immunocompromised patients.

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Section: Aid In Mdsmentioning

confidence: 98%

Section: Aid In Mdsmentioning

confidence: 99%

Section: Aid In Mdsmentioning

confidence: 99%

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Autoimmune Complications in Hematologic Neoplasms

Barcellini

Giannotta

Fattizzo

2021

Cancers

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“…Associations between phenotype and genotype have been recently identified within SIADs that are associated with MDS. Wesner et al reported a French retrospective caseseries with a literature review of 39 patients with Behçet's-like syndrome associated with trisomy-8 positive MDS, characterized by orogenital aphtosis, skin features and severe ulcerative digestive disease of ileocecal distribution [55]. Other clinical manifestations of Behçet's disease, such as arthritis or neutrophilic dermatosis, have also been described in MDS patients with trisomy 8, with the exception of ocular and neurologic manifestations that are usually absent.…”

Section: Characteristics Of the Underlying Mdsmentioning

confidence: 99%

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Jachiet

Fenaux

Sevoyan

et al. 2021

Hemato

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Systemic auto-inflammatory or autoimmune diseases (SIADs) develop in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML). With or without the occurrence of SIADs, the distribution of MDS subtypes and the international or CMML-specific prognostic scoring systems have been similar between MDS/CMML patients. Moreover, various SIADs have been described in association with MDS, ranging from limited clinical manifestations to systemic diseases affecting multiple organs. Defined clinical entities including systemic vasculitis, connective tissue diseases, inflammatory arthritis and neutrophilic diseases are frequently reported; however, unclassified or isolated organ impairment can also be seen. Although the presence of SIADs does not impact the overall survival nor disease progression to acute myeloid leukemia, they can help with avoiding steroid dependence and make associated adverse events of immunosuppressive drugs challenging. While therapies using steroids and immunosuppressive treatment remain the backbone of first-line treatment, increasing evidence suggests that MDS specific therapy (hypomethylating agents) and sparing steroids may be effective in treating such complications based on their immunomodulatory effect. The aim of this review was to analyze the epidemiological, pathophysiological, clinical and therapeutic factors of systemic inflammatory and immune disorders associated with MDS.

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“…Until now, however, no paper has reported hematologic involvement as a particular phenotype in BS, and there has been little research on hematologic involvement in large BS samples. Some studies have even referred to it as a "Behçet-like disease" [19,20]. Analyses of several case reports have shown an association between trisomy 8 and intestinal BD with MDS [2,3,21,22].…”

Section: Discussionmentioning

confidence: 99%

Clinical Association Between Behçet Syndrome, Cytopenia, and Acquired Trisomy 8: a New Clinical Syndrome?

Yan¹,

Luo²,

Ma³

et al. 2021

Preprint

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Objectives: Behçet syndrome (BS) is heterogeneous with clinical variability across ethnicities and geographic locations. This study analyzed the clinical characteristics of BS associated with cytopenia and trisomy 8.Methods: This was a retrospective study of Chinese BS patients in the Shanghai Behçet’s disease database who were diagnosed with cytopenia and trisomy 8 between October 2012 and April 2019. All subjects fulfilled the International Study Group (ISG) criteria or the International Criteria for Behçet’s Disease (ICBD).Results: Twenty-one patients with BS had cytopenia with trisomy 8; 16 (16/21, 76.2%) were diagnosed with myelodysplastic syndrome (MDS). Compared with 1,147 patients with BS without cytopenia, patients with BS and trisomy 8/cytopenia were more likely to be female (23.8% vs 51.8%,p= 0.011), older (37.9 ± 12.8 vs 45.1 ± 12.0, p = 0.007), have fewer skin lesions (33.3% vs 56.7%,p= 0.033), fever (52.4% vs 1.1%, p < 0.001), have higher C-reactive protein, have higher erythrocyte sedimentation rate (ESR), and display a higher rate of gastrointestinal (GI) ulceration (61.9% vs 9.2%, p < 0.001). By contrast, they had a lower leukocyte count, lower hemoglobin levels, and a lower platelet count (p < 0.05 for all comparisons). Ulcers in the ileocecal region were more frequently seen in intestinal patients with BS with trisomy 8 than in patients with BS without cytopenia (100.0% vs 41.8%; p = 0.002). Compared with 54 patients with MDS without BS symptoms, those with BS and trisomy 8-cytopenia were more likely to be female (23.8% vs 70.4%, p< 0.001) and younger (37.9 ± 12.8 vs 67.2 ± 12.4, p= 0.001). In the follow-up, three patients with BS with trisomy 8 (3/21, 14.3%) died, which is a higher rate than in patients with BS (5/1147, 0.44%) and much lower than in patients with MDS (13/54, 24.1%).Conclusions: The data on patients with BS with cytopenia and trisomy 8 were different from BS or MDS in epidemiology, clinical performance, and prognosis. BS with cytopenia and acquired trisomy 8 may be a new clinical syndrome.

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Gastrointestinal Behcet’s-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review

Cited by 28 publications

References 25 publications

Autoimmune Complications in Hematologic Neoplasms

Autoimmune Complications in Hematologic Neoplasms

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

Clinical Association Between Behçet Syndrome, Cytopenia, and Acquired Trisomy 8: a New Clinical Syndrome?

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