Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

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Bone marrow biopsy is a diagnostic tool largely used in the evaluation of a broad number of disorders that could affect the hematopoietic system. Differently, bone marrow aspirate clot technique is rarely performed even though it has been described in literature. Here, we highlight the utility of the bone marrow aspirate clot, exemplifying through the discussion of three clinical cases in which this technique was used for diagnosis and follow-up purposes: megaloblastic hemopathy, multiple myeloma, and chronic lymphocytic leukemia. Bone marrow clot analysis increases sensitivity to diagnose hemopathies and offers the possibility of morphological evaluation and anatomopathological study, with the advantage of not needing decalcification processes, hence improving antigenic expression in immunohistochemical and FISH techniques. It is an easy-to-perform technique, offering a quick, reliable, and more comfortable procedure for patients.

IntroductionMultiple myeloma (MM) is an incurable malignant neoplasm that accounts for approximately 1% of all cancers and 10% of haematological malignancies. Bortezomib is one of the most commonly used medications in first-line treatment and subsequent relapses, either as a single agent or in combination with other therapies. This study aims to assess the effects of bortezomib on the overall survival (OS), progression-free survival, overall response rate, time to next treatment, health-related quality of life, compliance, adverse events and treatment-related death in patients with MM.Methods and analysisWe have performed a systematic review and meta-analysis and will include both randomised and non-randomised controlled studies where the effect of bortezomib was compared in similar or dissimilar background therapies in each arm. General and adaptive search strategies have been created for the following electronic health databases: Embase, Medline, LILACS and CENTRAL. Two reviewers have independently selected eligible studies, will assess the risk of bias, and will extract data from the included studies. Similar outcomes will be plotted in the meta-analysis using the Stata Statistical Software V.17. The relative risk will be calculated with a 95% CI as the effect size of bortezomib. For the OS and progression-free survival, we calculate the overall OR from the HRs of each included study. Peto’s one-step OR will be calculated for event rates below 1%. We will use the Grading of Recommendations Assessment, Development and Evaluation system to evaluate the certainty of evidence.Ethics and disseminationAs no primary data collection will be undertaken, formal ethical assessment is not required. We plan to present the results of this systematic review in a peer-reviewed scientific journal, conferences and popular press.PROSPERO registration numberCRD42020151142.

Objective: To report the outcomes of a systematic literature review of guidelines and consensus on the management of paroxysmal nocturnal hemoglobinuria (PNH) and describe the main therapeutic options available worldwide. Methods: A systematic literature review was conducted in April 2018 with no time limit and reported in line with the PRISMA statement. The AGREE II instrument was used to determine the quality of each guideline included in the systematic review. Results: Eight guidelines/consensus were eligible, one developed by an international group, two in Spain, and one each in Turkey, Germany, Argentina, Australia and the United Kingdom. Supportive treatment with erythrocyte transfusion, anticoagulants and steroids is indicated by all guidelines and consensus. The use of erythropoietin is suggested by three of them. Recommendations for the prescription of eculizumab were consistent in all but one guideline, published in 2005. Allogeneic hematopoietic stem cell transplantation is reported as the only potentially curative treatment for PNH, although its association with high mortality and morbidity rates is emphasized, being indicated for a selected group of patients. The AGREE II scores applied for each domain showed in general a low and heterogeneous methodological quality among guidelines. Conclusion: Despite the low and heterogeneous methodological quality, in general the comparison of guidelines and consensus for PNH management showed consistent recommendations regarding supportive care, eculizumab and hematopoietic stem cell transplantation.