Longstanding Remission of Adult Onset Still’s Disease Under Imatinib Therapy in a Patient with Chronic Myelogenous Leukemia: Figure 1.

Nagasaki, Yoji; Miyamoto, Toshihiro; Henzan, Hideho; Nagafuji, Koji; Harada, Mine; Akashi, Koichi

doi:10.3899/jrheum.081159

Cited by 6 publications

(2 citation statements)

References 11 publications

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“…specimens are nondiagnostic of lymphoma at the time of initial presentation 40,43 to account for a paraneoplastic presentation and a diagnosis of AOSD is rendered. 26,31,40,43 To a lesser degree, leukemias and myelodysplastic syndrome have also been linked to AOSD-like presentations, 23,24,35,36,53 some not presenting until 6 years after the initial diagnosis of AOSD despite a normocellular marrow biopsy specimen at the time of initial presentation. 23 The second strongest association for AOSD-like symptomatology is with breast cancer (6/31; 19%).…”

Section: Anamentioning

confidence: 98%

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Sun

Brezinski

Berliner

et al. 2015

Journal of the American Academy of Dermatology

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Section: Anamentioning

confidence: 98%

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Sun

Brezinski

Berliner

et al. 2015

Journal of the American Academy of Dermatology

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“…About 10 to 20% of MDS/CMML could be associated with various extra hematological features, e.g., neutrophilic dermatoses, systemic vasculitis, inflammatory arthritis, pseudo-Behçet’s disease, and relapsing polychondritis [ 5 , 6 , 7 , 8 ]. For years now, the state of autoinflammatory disorders associated with neoplasia reported in the literature were mainly associated with solid cancer but also MDS/CMML, myeloproliferative disorders, and lymphoma, and identified in the literature as “paraneoplastic AOSD” or “malignant associated AOSD” [ 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 ].…”

Section: Introductionmentioning

confidence: 99%

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

Delplanque

Aouba

Hirsch

et al. 2021

JCM

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Background: Patients with solid cancers and hematopoietic malignancy can experience systemic symptoms compatible with adult-onset Still’s disease (AOSD). The newly described VEXAS, associated with somatic UBA1 mutations, exhibits an overlap of clinical and/or biological pictures with auto inflammatory signs and myelodysplastic syndrome (MDS). Objectives: To describe a cohort of patients with signs of undifferentiated systemic autoinflammatory disorder (USAID) concordant with AOSD and MDS/chronic myelomonocytic leukemia (CMML) and the prevalence of VEXAS proposed management and outcome. Methods: A French multicenter retrospective study from the MINHEMON study group also used for other published works with the support of multidisciplinary and complementary networks of physicians and a control group of 104 MDS/CMML. Results: Twenty-six patients were included with a median age at first signs of USAID of 70.5 years with male predominance (4:1). Five patients met the criteria for confirmed AOSD. The most frequent subtypes were MDS with a blast excess (31%) and MDS with multilineage dysplasia (18%). Seven patients presented with acute myeloid leukemia and twelve died during a median follow-up of 2.5 years. Six out of 18 tested patients displayed a somatic UBA1 mutation concordant with VEXAS, including one woman. High-dose corticosteroids led to a response in 13/16 cases and targeted biological therapy alone or in association in 10/12 patients (anakinra, tocilizumab, and infliximab). Azacytidine resulted in complete or partial response in systemic symptoms for 10/12 (83%) patients including 3 VEXAS. Conclusion: Systemic form of VEXAS syndrome can mimic AOSD. The suspicion of USAID or AOSD in older males with atypia should prompt an evaluation of underlying MDS and assessment of somatic UBA1 mutation.

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Pseudomaladie de Still et néoplasie : une observation de mélanome et revue de la littérature

Liozon

Vidal-Cathala

et al. 2014

La Revue de Médecine Interne

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Longstanding Remission of Adult Onset Still’s Disease Under Imatinib Therapy in a Patient with Chronic Myelogenous Leukemia: Figure 1.

Cited by 6 publications

References 11 publications

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

USAID Associated with Myeloid Neoplasm and VEXAS Syndrome: Two Differential Diagnoses of Suspected Adult Onset Still’s Disease in Elderly Patients

Pseudomaladie de Still et néoplasie : une observation de mélanome et revue de la littérature

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