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Brescia, Italy

This study aimed to assess the von Willebrand factor (VWF) antigen levels and its proteolytic enzyme ADAMTS13 activity in preeclampsia. The study includes 10 non-pregnant women, 50 normal pregnancy, and 110 preeclamptic (PE) women at the same period of pregnancy. For all studied groups plasma ADAMTS13 activities were determined with the FRETs-VWF 73 assay, while VWF antigen levels with an immunoturbometric assay. The plasma ADAMTS13 activity was significantly reduced in PE as compared with normal pregnancy and non-pregnant women (P < 0.01 for both). In contrast, plasma VWF antigen and VWF RCO were significantly elevated in PE, as compared with normal pregnancy group as well as non-pregnant women (P < 0.01 for both). In conclusion, reduction of plasma ADAMTS13 and elevation in VWF might have a role in the pathogenesis of PE. © W. S. Maney & Son Ltd 2013.

Tadmor T.,Hematology Unit | Polliack A.,Hebrew University of Jerusalem
Leukemia and Lymphoma | Year: 2011

Unusual clinical manifestations, rare sites of involvement, and associations with other disorders and malignancies occurring in patients with hairy cell leukemia (HCL) are uncommon events encountered in a relatively rare disease. The exact prevalence of these associations is difficult to determine accurately in HCL as they are often anecdotal case reports and not always detailed in all larger series of patients. This short review deals with the unusual clinical manifestations and rare sites of involvement of the disease and lists some of the disorders associated with HCL, based on what has been reported in the literature as well as from personal experience. No attempts are made here to establish the true prevalence of these phenomena and only selected references are included. Some details of the coexistence of HCL with other neoplasias, hematological disorders, and 'paraneoplastic' autoimmune disorders are provided, while opportunistic infections in HCL, particularly atypical mycobacterial disease, are briefly discussed. For the sake of brevity many of the details are provided in tabular form. © 2011 Informa UK, Ltd.

Dolberg O.J.,Hematology Unit | Dolberg O.J.,Tel Aviv University | Levy Y.,Meir Medical Center | Levy Y.,Tel Aviv University
Autoimmunity Reviews | Year: 2014

Aplastic anemia (AA) is a disease characterized by pancytopenia and hypoplastic bone marrow caused by the decrease of hematopoietic stem cells. The pathogenesis of AA is complex and involves an abnormal hematopoietic microenvironment, hematopoietic stem cell/progenitor cell deficiencies and immunity disorders. Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. Herein, we will update the main issues concern AA according to our literature review. © 2014 Elsevier B.V.

Iolascon A.,CEINGE | Iolascon A.,University of Naples Federico II | Heimpel H.,University of Ulm | Wahlin A.,Umea University | And 2 more authors.
Blood | Year: 2013

The congenital dyserythropoietic anemias (CDAs) are hereditary disorders characterized by distinct morphologic abnormalities of marrow erythroblasts. The unveiling of the genes mutated in the major CDA subgroups (I-CDAN1 and II-SEC23B) has now been completed with the recent identification of the CDA III gene ( KIF23). KIF23 encodes mitotic kinesin-like protein 1, which plays a critical role in cytokinesis, whereas the cellular role of the proteins encoded by CDAN1 and SEC23B is still unknown. CDA variants with mutations in erythroid transcription factor genes (KLF1 and GATA-1 ) have been recently identified. Molecular diagnosis of CDA is now possible in most patients. © 2013 by The American Society of Hematology.

Niscola P.,Hematology Unit
Expert Review of Hematology | Year: 2010

Mucosal barrier injury (MBI), also known as mucositis, is the result of the cytotoxic effects of many treatments given for hematological malignancies (HMs) and represents a major source of potentially devastating clinical complications and negative consequences afflicting the patients management, such as a longer hospitalization, the need of analgesic and total parenteral nutrition use, and increased costs. The available measures for the prevention and treatment of MBI have been substantially limited to the control of pain, infection, bleeding and nutrition. However, in the last decade, a better insight into the complex pathogenesis of MBI has led to the development of novel therapeutic options, such as palifermin, which has been one of the major breakthroughs in the management of this condition, potentially allowing a targeted approach to MBI. Nevertheless, and despite these significant advances, MBI still remains a significant clinical problem in the management of HM and an important burden of sufferance for afflicted patients. © 2010 Expert Reviews Ltd.

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