Anaplastic large cell lymphoma in a patient with MAGIC syndrome: a case and review of the literature
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CASE BASED REVIEW
Anaplastic large cell lymphoma in a patient with MAGIC syndrome: a case and review of the literature Zhe Chen 1,2 & Shangzhu Zhang 1 & Li Wang 1 & Yunyun Fei 1 & Min Shen 1 & Huanwen Wu 3 & Jinjing Liu 1
& Wenjie Zheng
1
Received: 18 May 2020 / Revised: 26 August 2020 / Accepted: 31 August 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract Mouth and genital ulcer with inflamed cartilage syndrome (MAGIC syndrome) is a rare autoinflammatory disorder with unknown etiology. Except for the common clinical manifestations mimicking Behçet’s disease and relapsing polychondritis, some other clinical entities are occasionally observed. In this report, we present a case in which a patient developed anaplastic large cell lymphoma 1 year after the diagnosis of MAGIC syndrome. Additionally, we review the clinical manifestations, management, and prognosis of MAGIC syndrome. Keywords Behçet’s disease . Lymphoma . MAGIC syndrome . Relapsing polychondritis
Introduction
Case presentation
MAGIC syndrome (mouth and genital ulcer with inflamed cartilage) is an autoinflammatory disorder first reported by Firestein et al. in 1985 [1]. It has been regarded as the overlap of Behçet’s disease (BD) and relapsing polychondritis (RP). The MAGIC syndrome is a rare clinical entity. Here, we present the case of a 25-year-old female who developed lymphoma 1 year after MAGIC syndrome was diagnosed. To the best of our knowledge, this is the first case report of lymphoma in patients with MAGIC syndrome. Informed consent was obtained from the patient’s legal representative.
The patient developed recurrent oval-shaped mouth ulcers in 2000 when she was ten. Mouth ulcers were accompanied by genital ulcers occasionally. In 2009, she was diagnosed as uveitis, and despite the treatment with prednisolone acetate ophthalmic solution, her symptoms waxed and waned. In August 2011, she presented with fevers, exacerbated mouth and genital ulcers, erythema nodosa, and superficial phlebitis in the lower extremities. Laboratory tests showed elevated ESR (81 mm/h) and CRP (8.2 mg/L). The autoantibodies (including anti-nuclear antibody (ANA), antineutrophil cytoplasmic antibodies (ANCA), and antiphospholipid antibody (aPL)) were all negative. The diagnosis of BD was made according to the 1990 International Study Group (ISG) criteria [2], and her symptoms slightly improved after prednisone and thalidomide therapy. Thalidomide was replaced by cyclosporine A (CsA) and cyclophosphamide (CTX) as she developed peripheral neuropathy 8 months later. She was referred to our department in May 2013 due to refractory mucosal ulcers and deteriorated vision impairment. Laboratory tests demonstrated decreased WBC level and normal ESR and CRP, whereas ophthalmologic examinations revealed active uveitis. A fter ruling out latent infection, CTX was discontinued, and infliximab (IFX) was introduced together with CsA (150 mg/d) and prednisone (40 mg/d initially, tapered gradually). Her mouth and genital ulcers healed, and vision im
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