The authors report a rare association: Hashimoto's thyroiditis, thrombocytopenic idiopathic purpura (Werlhof's disease) and Steinert's myotonic dystrophia. The authors outline the fingerprints of each disease, report particular syndromes and compare that association to data of partially reviewed literature. They suppose a suggestive pathogenetic connection among these diseases, due to the coexistence of altered genetic, endocrine and autoimmune factors and consequently impaired cell membrane permeability.

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Background And Aims:  Immune thrombocytopenic purpura (ITP) is an acquired bleeding disorder characterized by autoantibodies against platelets. The clinical presentation is variable; the main symptom is bleeding, and many patients are asymptomatic; others have nonspecific symptoms like fatigue. Uncommonly, ITP can present with paradoxical thrombosis.

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Eltrombopag is an orally administered, non-peptide, thrombopoietin receptor agonist which initiates thrombopoietin signaling and stimulates the production of normally functioning platelet. We aimed to do a systematic review and meta-analysis of currently available published data to verify whether eltrombopag treatment in patients with chronic immune-mediated thrombocytopenia can prolong survival. We searched for published, randomized, controlled trials in PubMed, Cochrane and Scopus databases using the following search strategy ("Eltrombopag" OR "Benzoates" OR "Hydrazines") AND ("Idiopathic Thrombocytopenic Purpura" OR "immune thrombocytopenia" OR "Idiopathic Thrombocytopenic Purpuras" OR "Immune Thrombocytopenia" OR "Autoimmune Thrombocytopenia" OR "Werlhof").

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[73-year-old man with refractory idiopathic thrombocytopenia purpura].

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ITP: a historical perspective.

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May 2011

Department of Haematology, St George's Hospital, Blackshaw Road, London, UK.

A clinical syndrome of bleeding and purpura consistent with a diagnosis of immune thrombocytopenia (ITP) was described by Werlhof long before platelets were identified as the cellular component of blood playing an essential role in primary haemostasis. Although a role for the spleen was suggested nearly a century ago, the pathophysiology of ITP has remained elusive for many decades. During this time Werlhof's disease was renamed idiopathic thrombocytopenic purpura, from which the acronym ITP originally derives.

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On the 3rd day following surgery to repair an incisional hernia, a 67-year-old male patient with Werlhof's disease (idiopathic thrombocytopenic purpura) was diagnosed with a histologically confirmed pyoderma gangraenosum (PG), a rare complication of wound healing. Dexamethasone pulse therapy resulted in rapid remission of the skin lesions. Further improvement was slowed when the patient suffered multiple organ failure in the intensive care unit, delaying his transfer to rehabilitation for 8 weeks.

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