Bone Marrow Involvement in a Patient with Alpha Heavychain Disease: Response to Tetracycline Treatment

A 28-year-old man from East Mediterranean area admitted with abdominal pain, weight loss and diarrhea. Barium x-ray studies showed segmentation, dilatation of bowel loops, mucosal folds thickening and delayed intestinal transit. Histological examination of biopsy specimens revealed villous atrophy and plasmacytic infiltration limited to mucosa and submucosa. Computed tomography showed multiple lymphadenopathy in the abdomen. Serum protein electropheresis and immunoelectropheresis indicated elevated IgA concentration. Bone marrow aspiration and biopsy revealed presence of lymphoplasmacytic infiltration. Immunohistochemical analysis of the intestine, lymph nodes showed positivity for CD45, CD-79, CD-20. After tetracycline treatment the patient’s symptoms, abdominal lymphadenopathy and bone marrow infiltration disappeared and IgA concentration decreased to normal levels

Lenalidomide-related malar rash-like lesion in patient with 5q-syndrome

It has become important to be careful in terms of side effects with the increased lenalidomide use. The most commonly reported adverse events with the use of lenalidomide were hematologic toxicities and less frequently peripheral edema, infections, gastrointestinal disorders, fatigue, and muscle cramps. In particular, the nonserious rash is the most common cause of permanent discontinuation of lenalidomide. Although morbilliform, urticarial, or acneiform rashes have been observed with lenalidomide, malar rash-like lesions have not been described previously. Our aim is to present here 71-year-old female myelodysplastic syndrome patient with deletion of 5q abnormality who should be discontinued lenalidomide due to recurrent skin reaction like a malar rash

Scientific people named in the classification of vasculitis

Abstract The first International Chapel Hill Consensus Conference was held in 1994. There have been suggestions about the nomenclature of systemic vasculitis. Important categories were added to the classification of vasculitis, and many changes were made for disease names in the second Chapel Hill Consensus Conference 2012, which were not included in the Chapel Hill Consensus Conference 1994. The new nomenclature was introduced instead of being referred to by many names such as Churg-Strauss and Wegener"s. New categories such as Behçet"s and Cogan etc. were also added. These people are honored by the classification. They contribute to science through their case studies, scientific articles, and observations. This article reviews only eponyms present in the current classification of vasculitis. The aim of this paper is to give information about scientists mentioned in the classification of vasculitis

Rhupus syndrome and Chiari's network

A 69-year-old female patient was admitted to our clinic with photosensitivity, symmetric erosive polyarthritis, and cutaneous vasculitis of lower extremities. Rhupus syndrome was diagnosed, and Chiari's network in the right atrium and interatrial septum patent foramen ovale was achieved on transthoracic and transesophageal echocardiography. If it is thought that increased prevalence of antiphospholipid antibodies in patients with rhupus, this congenital remnant is important for the thrombosis risk, cardiac event, and stroke. The association of both diseases may lead to more serious events and cause worse prognosis. Here, our aim is to present a 69-year-old female patient with rhupus syndrome presenting with cutaneous vasculitis and Chiari's network in the right atrium

Anti-interleukin-1 treatment among patients with familial Mediterranean fever resistant to colchicine treatment. Retrospective analysis

ABSTRACT BACKGROUND: Up to 5% of familial Mediterranean fever (FMF) cases are unresponsive to colchicine, through resistance, side effects and toxicity. Anakinra is an alternative treatment for FMF patients whose disease remains uncontrolled with colchicine. We aimed to evaluate anti-interleukin-1 treatment regarding clinical findings, laboratory parameters and quality of life (QoL) among FMF patients presenting resistance and toxicity towards colchicine. DESIGN AND SETTING: Descriptive observational study at the rheumatology clinic, Adnan Menderes University Medical School, Aydın, Turkey. METHODS: Among the patients included, age, sex, MEFV genotypes, acute-phase reactants, hepatic/renal function tests, average colchicine dose, disease duration, attack frequency, attack duration, disease severity, proteinuria, amyloidosis and QoL were evaluated. Colchicine resistance was defined as > 6 typical episodes/year or > 3 per 4-6 months. Kolmogorov-Smirnov, Friedman and two-way analysis of variance tests were used for statistical analyses. RESULTS: Between 2015 and 2017, 14 FMF patients receiving anakinra were enrolled. The mean colchicine dose was 1.7 ± 0.3 mg/day before use of anakinra. Ten patients were attack-free after treatment, while three showed reductions of at least 50% in attack frequency, attack duration and disease severity. Proteinuria levels in all patients with renal amyloidosis decreased after treatment. QoL among patients with renal amyloidosis differed significantly from QoL among non-amyloidosis patients. Mean visual analogue scale scores significantly improved in both groups after use of anakinra. CONCLUSIONS: Use of anakinra reduced attack frequency and proteinuria and acute-phase reactant levels, and improved QoL, with only a few uncomplicated side effects among colchicine-resistant or intolerant FMF patients. Injection-site reactions of severity insufficient to require discontinuation of treatment were seen

The relationship between blood eosinophil count and disease activity in ankylosing spondylitis patients treated with TNF-α inhibitors

Aim: Anti-tumor necrosis factor-alpha (Anti-TNF-α) therapy has achieved an important position, are widely used for ankylosing spondylitis (AS) patients. TNF-α inhibition improves clinical outcomes and has differential effects on haematopoiesis. Information about effects on eosinophils is limited. The aim of our study is to determine the relationship between blood eosinophil counts in AS patients treated with TNF-α inhibitors. Methods: Seventy-five patients diagnosed with AS according to modified New York criteria were enrolled in this study. Disease activity was assessed by BASDAI, and erythrocyte sedimentation rate, C-reactive protein of patients were evaluated. All data were analyzed with Spearman’s correlation and Friedman's Two-Way by using SPSS version 19.0 statistical software, and p  0.05). It counts lower before anti-TNF-α therapy compare with post-treatment (130/mm3, 140/mm3 and 190/mm3, respectively). There was no statistically significant difference between pre- and 3rd month (p > 0.05), while correlation was found between pre- and 6th month, and also 3rd month and 6th month (p < 0.001, p = 0.002, respectively) for blood eosinophil count. Conclusion: To the best of our knowledge, our study is the first to evaluate blood eosinophil counts and disease activity with anti-TNF-α therapy. Blood eosinophil count may be affected by TNF-α inhibition in patients with AS. Keywords: Ankylosing spondylitis, Eosinophil, Tumor necrosis factor-

The relationship between platelet distribution width and disease activity in patients with polymyositis

Introduction Muscle enzymes are an indicator of ongoing muscle damage and disease activity in patients with idiopathic inflammatory myopathy. Although platelet-related parameters have been shown to be useful as markers of disease activity in autoimmune diseases, the relationship between platelet distribution width (PDW) and disease activity has not been previously studied in polymyositis. We aimed to determine the relationship between PDW and disease activity in patients with polymyositis. Material and methods Twenty-seven patients with polymyositis and thirty healthy controls were included in the study. Disease activity was evaluated using the myositis disease activity assessment Visual Analogue Scale (MYOACT) and the Myositis Intention to Treat Index (MITAX). The relationship between PDW and disease activity was evaluated using Pearson’s or Spearman’s correlation and reliability was assessed using correlation coefficients. Results The mean platelet volume (MPV) and plateletcrit (PCT) were significantly higher and PDW was significantly lower in patients with polymyositis compared to the control group. The mean PDW levels were lower in patients with constitutional symptoms and arthralgia/arthritis (p < 0.005). Although PDW levels were lower in patients with mechanical hand, lung involvement, or dysphagia compared to patients without, there was no statistically significant difference between them. Platelet distribution width was found to be negatively correlated with disease activity. Conclusions We found that PDW was negatively correlated with MYOACT and MITAX, widely used tools in assessing the disease activity of polymyositis. Based on this, PDW may be utilized as a non-invasive potential index to assess disease activity in patients with polymyositis