Serum Vitamin D Levels in Children With Newly Diagnosed and Chronic Immune Thrombocytopenia
The primary objective of the study was to assess the vitamin D (VD) status of patients suffering from ITP. Children from the case cohort (total 21) were recruited from chronic ITP patients (followed as outpatients) and newly diagnosed ITP (prospective study) patients. VD deficiency (values below 75 nmol/L) was detected in 11 patients with newly diagnosed ITP, and 7 patients with chronic ITP. Only three patients with newly diagnosed, and none with chronic ITP had normal VD values. Newly diagnosed ITP patients had statistically significantly higher values (p (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 10, 2016 Category: Hematology Authors: Srđana Čulić, Joško Markić, Davor Petrović, Paško Konjevoda, Jasminka Pavelić Source Type: research

Anti-D Treatment for Pediatric Immune Thrombocytopenia: is the Bad Reputation Justified?
The purpose of this study is to assess the efficacy and side effect profile of the repeated use of anti-D for the treatment of pediatric immune thrombocytopenia (ITP) in a large pediatric hematology center. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 7, 2016 Category: Hematology Authors: Joanne Yacobovich, Sabreen Abu-Ahmed, Orna Steinberg-Shemer, Tracie Goldberg, Miriam Cohen, Hannah Tamary Source Type: research

Imbalanced immune homeostasis in immune thrombocytopenia
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder resulting from low platelet counts caused by inadequate production as well as increased destruction by autoimmune mechanisms. As with other autoimmune disorders, chronic ITP is characterized by perturbations of immune homeostasis with hyperactivated effector cells as well as defective regulatory arm of the adaptive immune system, which will be reviewed here. Interestingly, some ITP treatments are associated with restoring the regulatory imbalance, although it remains unclear whether the immune system is redirected to a state of tolerance once treatment is dis...
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Karina Yazdanbakhsh Source Type: research

Thrombopoietin receptor agonists: a new immune modulatory strategy in immune thrombocytopenia?
In 2008, new drugs that mimic the effects of thrombopoietin became available for the treatment of primary immune thrombocytopenia, eg, romiplostim and eltrombopag. These drugs activate the thrombopoietin receptor, stimulate the production of megakaryocytes, and increase the production of platelets. Important clinical observation has been gained, such as unexpected long-term remission after stopping thrombopoietin receptor agonists. The pathophysiology of this unforeseen cure is currently the subject of discussion and is investigated in clinical trials and laboratory research projects. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Alexandra Schifferli, Thomas Kühne Source Type: research

Immunomodulation and immune thrombocytopenia: some unmet needs, questions, and outlook
During the last two decades, new therapeutic strategies have been developed, particularly anti-CD20 agents and thrombopoietin-receptor (TPO-r) mimetics, for immune thrombocytopenia (ITP). However, although the new efficient drugs have deeply modified the therapeutic strategy and the disease prognosis, there are still unmet needs and challenges. Concerning rituximab, reassuring data concerning its safety have recently been reported. The main limitation of the treatment is its modest long-term efficacy, with frequent disease relapse. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Bertrand Godeau Source Type: research

Viruses, anti-viral therapy, and viral vaccines in children with immune thrombocytopenia
Immune thrombocytopenia (ITP) might be preceded by silent or overt viral infections. Similarly, anti-viral drugs and viral vaccines could also trigger ITP and might play a central role in its pathogenesis. The seasonal nature of childhood ITP suggests that viral infections might initiate immune responses that increase the predisposition and occurrence of ITP. Active cytomegalovirus or Epstein-Barr virus should be considered in differential diagnosis when thrombocytopenia is associated with lymphadenopathy, especially with splenomegaly. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Mohsen S. Elalfy, Diane Nugent Source Type: research

From Immune Substitution to Immunomodulation
Intravenous immunoglobulins (IVIG) are currently used in many fields of medicine for replacement and immunomodulation. This review focusses on the milestones in the history of human immunoglobulins since the initial observation by Ogden C. Bruton who described replacement therapy in a single boy with agammaglobulinemia. Since then, the preparations used for treatment have been markedly improved with respect to tolerability, clinical efficacy and pathogen safety. Preparations and appropriate pumps for s.c. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Volker Wahn Source Type: research

Imbalanced Immune Homeostasis in Immune Thrombocytopenia (ITP)
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder resulting from low platelet counts caused by inadequate production as well as increased destruction by autoimmune mechanisms. As with other autoimmune disorders, chronic ITP is characterized by perturbations of immune homeostasis with hyperactivated effector cells as well as defective regulatory arm of the adaptive immune system which will be reviewed here. Interesting some ITP treatments are associated with restoring the regulatory imbalance, although it remains unclear whether the immune system is re-directed to a state of tolerance once treatment is discon...
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Karina Yazdanbakhsh Tags: April 2016 Suppl 1 ICIS Source Type: research

Thrombopoietin Receptor Agonists: A New Immune Modulatory Strategy in ITP?
In 2008, new drugs that mimic the effects of thrombopoietin became available for the treatment of primary immune thrombocytopenia, e.g., romiplostim and eltrombopag. These drugs activate the thrombopoietin receptor, stimulate the production of megakaryocytes, and increase the production of platelets. Important clinical observation has been gained, such as unexpected long-term remission after stopping thrombopoietin receptor agonists. The pathophysiology of this unforeseen cure is currently the subject of discussion and is investigated in clinical trials and laboratory research projects. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Alexandra Schifferli, Thomas Kühne Tags: April 2016 Suppl 1 ICIS Source Type: research

Refractory Autoimmune Disease: An overview of when first-line therapy is not enough
A recent Intercontinental Cooperative ITP Study Group (ICIS) meeting in September 2015 focused on immunomodulation across the spectrum of autoimmune conditions. It became clear to the attendees that in this wide range of conditions, there is a subset of patients that remain highly refractory to first line therapy. Therapeutic approaches to these patients vary greatly and while many different immunomodulatory agents have been investigated, few have seen universal success. We outline here the landscape of immunomodulation therapy for refractory patients across a variety of autoimmune conditions in order to highlight the vari...
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Cindy Neunert, Roula Farah, Joanne Yacobovich, Ellis Neufeld Tags: April 2016 Suppl 1 ICIS Source Type: research

Immunomodulation and Immune Thrombocytopenia: Some Unmet Needs, Questions and Outlook
During the last 2 decades, new therapeutic strategies have been developed, particularly anti-CD20 agents and thrombopoietin-receptor (TPO-r) mimetics, for immune thrombocytopenia (ITP). However, although the new efficient drugs have deeply modified the therapeutic strategy and the disease prognosis, there are still unmet needs and challenges. Concerning rituximab, reassuring data concerning its safety has recently been reported. The main limitation of the treatment is its modest long-term efficacy, with frequent disease relapse. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Bertrand Godeau Tags: April 2016 Suppl 1 ICIS Source Type: research

Vaccinations and Secondary Immune Thrombocytopenia with Antiphospholipid Antibodies by Human Papillomavirus Vaccine
A 13-year-old female patient developed immune thrombocytopenic purpura (ITP) and concomitant positive antiphospholipid antibodies (aPL) following vaccination with a quadrivalent human papillomavirus (HPV) vaccine. During the course of a disease, she developed clinical manifestation with bleeding and she was treated with intravenous immunoglobulins. Consequently, the number of her platelets remained critically low and she was put on steroids and rituximab. Since then, her platelet count remain within the normal range, but her antiphospholipid antibodies are still present. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Mojca Bizjak, Or Bruck, Darja Kanduc, Sonja Praprotnik, Yehuda Shoenfeld Tags: April 2016 Suppl 1 ICIS Source Type: research

Current treatments and developments in pemphigoid diseases as paradigm diseases for autoantibody-driven, organ-specific autoimmune diseases
Pemphigoid diseases constitute a group of difficult-to-treat, remitting-relapsing autoimmune skin diseases. They are autoantibody-driven diseases with well-defined autoantigens in the hemidesmosomal complex at the dermal-epidermal junction (DEJ). Autoantibody deposition initiates the recruitment of granulocytes to the DEJ, where these cells degrade the hemidesmosomal complex, disrupting dermal-epidermal adherence, which clinically manifests as blisters and erosions. Due to their well-defined autoantigens and the location at the body surface, allowing the direct observation of inflammation throughout its course, pemphigoid ...
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Christian D. Sadik, Detlef Zillikens Tags: April 2016 Suppl 1 ICIS Source Type: research

Immunological treatment of Multiple Sclerosis
Treatment of Multiple Sclerosis (MS) has been a challenge since its first description by Charcot. The advent of immunomodulatory drugs in the mid 90ies brought the first big change in the treatment of MS patients. During the last 10 years there has been an ongoing tremendous evolution of novel treatment options for relapsing-remitting MS. These options include monoclonal antibodies, which inhibit migration of lymphocytes (natalizumab), deplete lymphocytes (alemtuzumab), or block the cytokine receptor IL-2 (daclizumab), teriflunomide that inhibits proliferation of activated lymphocytes, fingolimod that modulates the sphingo...
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Martin Diebold, Tobias Derfuss Tags: April 2016 Suppl 1 ICIS Source Type: research

Immunomodulation of rheumatologic disorders with non-biologic disease modifying antirheumtic drugs
Although biological agents have revolutionized the immunomodulation of many rheumatic disorders, conventional disease modifying antirheumatic drugs (DMARDs) remain important glucocorticosteroid sparing agents and combination partners. In rheumatoid arthritis, low-dose glucocorticosteroids can be regarded as a DMARD due to preventive effects on joint erosions. Therapy with methothrexate and possibly also other DMARDs may alter the natural evolution of rheumatoid arthritis severity over time and therapy should be instituted as early as possible. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Ulrich A. Walker Source Type: research