Accurate diagnosis and classification of von Willebrand disease are essential for optimal management. The von Willebrand factor multimers analysis is in the phenotypic classification, especially in discrepant cases, an integral part of the diagnostic process. The aim of this study was to evaluate the performance of a new Hydragel 11VWF multimer assay (H11VW). Results: Comparison study did not reveal any significant difference between H5VW and H11VW. The assessment of within-subject results, using H5VW and H11VW, demonstrates the reproducibility of the method in both healthy and VWD patients’ samples collected over time, with identical multimeric pattern on densitometric curves.Conclusion: This assay demonstrated acceptable performance, produced within-day results and can be used in routine practice for the visual investigation of gel and quantitative estimation of VWF multimer fractions.
Objective:Rare disease Background:Pseudolymphoma is a rare disorder that can mimic lymphoma both clinically and histologically. It usually affects middle-aged females. Since pseudolymphoma is a rare disorder not only is diagnosing the condition difficult, but there is also a lack of standardized treatment guidelines. In the literature, anti-CD20 monoclonal antibody rituximab is described as an effective treatment option. Case Report:46-year-old female fell ill suddenly with swelling and enlargement of her chin. Multiple skin biopsies were done, which were re-evaluated multiple times as well. Each ended with a new diagnosis for the patient. Finally, in the last revision of biopsy material, pseudolymphoma was confirmed. The patient received multiple courses of corticosteroid treatments -locally and systemically -without long lasting effect. After diagnosis of pseudolymphoma, the patient was started on intravenous rituximab and this treatment was effective. Conclusions:Cutaneous pseudolymphoma is a diagnostic challenge. Rituximab is a treatment option for refractory pseudolymphoma. Since there are no treatment guidelines for pseudolymphoma, more clinical studies are needed to establish best treatment options for these patients. Therefore, each reported clinical case is important.
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