Celiac disease with Evans syndrome and isolated immune thrombocytopenia in monozygotic twins: A rare association
Celiac disease is a multisystem immune-mediated disorder caused by exposure to dietary gluten in genetically predisposed individuals. The clinical presentation is characterized by a multitude and diversity of symptoms and complications. The coexistence of celiac disease with other autoimmune disorders has been established, most frequently with type 1 diabetes mellitus and autoimmune thyroiditis. The association of celiac disease with immune-mediated hematologic conditions has been rarely reported. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 6, 2016 Category: Hematology Authors: Jelena Roganovic Tags: April 2016 Suppl 1 ICIS Source Type: research

Viruses, anti-viral therapy and viral vaccines in children with immune thrombocytopenia
Immune thrombocytopenia 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 Bar 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, Nugent Diane Source Type: research

5th Intercontinental Cooperative ITP Study Group (ICIS) expert meeting in Fl üeli-Ranft, Switzerland, September 2015
The 5th Intercontinental Cooperative ITP Study Group (ICIS) expert meeting in Fl üeli-Ranft, Switzerland, September 24-26, 2015 was dedicated to immunomodulation in immune thrombocytopenia (ITP) and other autoimmune disorders. In particular we asked the question “How may biological immunomodulators influence the immune system?“ Wahn, Crow and Lazarus, Heitink et al, and van Laarhoven et al focused on the first such human derived immunomodulator, polyvalent immunoglobulin concentrate (IVIG), and Yacobovich et al on anti-D immunoglobulins. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Paul Imbach, Thomas K ühne Source Type: research

Immunomodulation of immunothrombocytopenia
Current treatment strategies in patients with immunothrombocytopenia (ITP) include immunosuppression and the stimulation of platelet production. Research over the last decade has emphasized the important role of Fc-receptors as key molecules responsible for autoantibody-mediated platelet depletion, allowing for a more specific targeting of this pathway instead of a generalized suppression of the immune system. This short review will discuss approaches aiming at interfering with key steps in this pathway, such as preventing the interaction of the platelet autoantibody immune complex with phagocytic cells, enhancing the clea...
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Falk Nimmerjahn Source Type: research

Mechanistic properties of intravenous immunoglobulin in murine immune thrombocytopenia: support for FcγRIIB falls by the wayside
Immune thrombocytopenia (ITP) is an autoimmune disorder characterised by platelet clearance resulting from the production of platelet-reactive autoantibodies. Platelet clearance appears to occur mainly via phagocytosis in the mononuclear phagocytic system, although T-cell–mediated platelet destruction, platelet apoptosis and dysregulation of platelet production can also play a role in disease pathogenesis. One of the most successful treatments for ITP is intravenous immunoglobulin (IVIg), and while it has been used in ITP for over 30 years, its mechanism(s) of action still remain unclear. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Andrew R. Crow, Alan H. Lazarus Source Type: research

5th Intercontinental Cooperative ITP Study Group (ICIS) expert meeting in Flüeli-Ranft, Switzerland, September 2015
The 5th Intercontinental Cooperative ITP Study Group (ICIS) expert meeting in Flüeli-Ranft, Switzerland, September 24-26, 2015 was dedicated to immunomodulation in immune thrombocytopenia (ITP) and other autoimmune disorders. In particular we asked the question „how may biological immunomodulators influence the immune system?“ Wahn, Crow and Lazarus, Heitink et al., and van Laarhoven et al. focused on the first such human derived immunomodulator, polyvalent immunoglobulin concentrate (IVIG), and Yacobovich et al. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Paul Imbach, Thomas Kühne Tags: April 2016 Suppl 1 ICIS Source Type: research

The Nonhemostatic Immune Functions of Platelets
Platelets are megakaryocyte-derived cellular fragments, which lack a nucleus and are the smallest circulating cells and are classically known to have a major role in supporting hemostasis. Apart from this well established role, it is now becoming evident that platelets are also capable of conveying other important functions, such as during infection and inflammation. This paper will outline these nonhemostatic functions in two major sections termed “Platelets versus pathogens” and “Platelet-target cell communication”. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Rick Kapur, John W. Semple Tags: April 2016 Suppl 1 ICIS Source Type: research

Immunomodulation of ITP
Current treatment strategies in patients with immunothrombocytopenia (ITP) include immunosuppression and the stimulation of platelet production. Research over the last decade has emphasized the important role of Fc-receptors as key molecules responsible for autoantibody mediated platelet depletion, allowing for a more specific targeting of this pathway instead of a generalized suppression of the immune system. This short review will discuss approaches aiming at interfering with key steps in this pathway, such as preventing the interaction of the platelet autoantibody immune complex with phagocytic cells, enhancing the clea...
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Falk Nimmerjahn Tags: April 2016 Suppl 1 ICIS Source Type: research

T cells in the pathogenesis of Immune Thrombocytopenia
Immune thrombocytopenia (ITP) is an acquired autoimmune bleeding disease characterized by both increased platelet destruction and reduced platelet production. ITP involves complicated upstream immune dysregulations in which T cell subsets are supposed to take the center stage. Survival of autoreactive T cells is prolonged. Th cell polarization is observed in Th1, Th17, and Th22 subsets with a positive correlation, whereas Tregs, a protective subset, is deficient in both frequency and function. Restoration of the imbalanced Th1/Th17/Tregs may correct the abnormal autoimmunity. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Yu Wei, Ming Hou Source Type: research

Mechanistic properties of IVIg in murine ITP: Support for FcγRIIB falls by the wayside
Immune thrombocytopenia (ITP) is an autoimmune disorder characterised by platelet clearance resulting from the production of platelet-reactive autoantibodies. Platelet clearance appears to occur mainly via phagocytosis in the mononuclear phagocytic system, although T cell mediated platelet destruction, platelet apoptosis and dysregulation of platelet production can also play a role in disease pathogenesis. One of the most successful treatments for ITP is intravenous immunoglobulin (IVIg), and while it has been used in ITP for over 30 years, its mechanism(s) of action still remain unclear. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Andrew R. Crow, Alan H. Lazarus Source Type: research

Immune Modulation for Autoimmune Disorders: Evolution of Therapeutics
Autoimmune disorders result from either congenital or acquired defects in central or peripheral immune tolerance. A genetic propensity may underlay the development of most such disorders, but an external trigger may be required for the eventual development of the autoimmune disease. The development of pharmacologic agents to treat such disorders by inducing self-tolerance has progressed over the last 60 years. Historically termed immunosuppressive agents, it is now understood that they may modulate the immune system in varied and unexpected ways. (Source: Seminars in Hematology)
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Howard A. Liebman Source Type: research

What do we Learn from Immunomodulation in Patients with Immune Thrombocytopenia?
Current therapeutic strategies for autoimmune diseases primarily rely on immunosuppression, but global immune suppression results in an increased risk for severe infection and malignancy. In contrast, immuomodulation is another therapeutic approach employing intrinsic or environmental regulators that exert modulatory effects by intervening multiple checkpoints of the immune system, leading to correction of dysregulated immune responses. We have learned that immunomodulation by intravenous immunoglobulin is highly efficacious and safe in patients with immune thrombocytopenia (ITP), an autoimmune disease mediated by IgG anti...
Source: Seminars in Hematology - April 5, 2016 Category: Hematology Authors: Masataka Kuwana Source Type: research

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Source: Seminars in Hematology - March 31, 2016 Category: Hematology Source Type: research

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Source: Seminars in Hematology - March 31, 2016 Category: Hematology Source Type: research

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Source: Seminars in Hematology - March 31, 2016 Category: Hematology Source Type: research