Werlhof

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. The risk of thrombosis in ITP may be higher than expected, which makes the management of ITP more challenging. This review aims to evaluate patients with ITP who develop thrombosis and identify potential risk factors related to thrombosis in this category of patients...

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")...

No abstract text is available yet for this article.

The use of point-of-care ultrasound (POCUS) and transesophageal echocardiography (TEE) in the perioperative and critical care setting is increasing worldwide. This increase has been driven by increasing educational opportunities and technologic advances. Nepal and Bangladesh are resource-limited countries where concerted efforts have been made to increase training in POCUS or TEE in the perioperative and critical care settings. This paper's focus is to present the current state of use and international efforts to improve education and skills in perioperative POCUS and TEE in Nepal and Bangladesh...

No abstract text is available yet for this article.

BACKGROUND: To show effectiveness of low-dose splenic irradiation in symptomatic congestive splenomegaly. METHODS: Five patients were referred to our department for symptomatic congestive splenomegaly within three years. Primary diseases were autoimmune hepatitis with liver cirrhosis (n=2), cystic fibrosis (n=1), granulomatous liver disease (n=1) and Werlhof disease with liver cirrhosis (n=1). Mean age was 54 years (range: 36-67). Patients received splenic irradiation with a total dose of 3 Gy (single dose: 0...

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...

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. Postoperative PG is always a differential diagnostic possibility in patients with sterile, progressive, painful, ulcerative skin lesions at or near the surgical wound...

No abstract text is available yet for this article.

In the first report of the concurrent immunodeficiency, thrombocytopenia, and eczema that we now call the Wiskott-Aldrich Syndrome (WAS), Alfred Wiskott asked whether it could be a familial form of Werlhof's disease (now called ITP). This review summarizes what is known about platelet production, consumption, and function in clinical and murine WAS. Both platelet production and consumption are affected by WASP deficiency. Likely molecular mechanisms have been identified for the former process, but remain problematic for the latter...

Ascorbic acid is a vitamin soluble in water and its deficiency in human body causes scurvy. Its symptoms in adults are gingivitis, susceptibility of blood vessels to damage and bleeding, changes in bones and cartilage and retarded wound healing. Ascorbic acid is necessary in redox processes taking place in cell. It is reversibly oxidized to dehydroascorbic acid and partially metabolized to inactive sulphide and oxalic acid, which is expelled in urine. It is well absorbed from the digestive system and easily reaches the tissues...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

UNLABELLED: Rituximab is a chimeric monoclonal antibody directed against normal and malignant mature B-lymphocytes and results in prolonged and severe B-cell depletion. Recently, rituximab has been successfully used in adult and paediatric disorders of B-lymphocytes such as autoimmune haemolytic anaemia and Werlhof disease. We report on two children with chronic immune thrombocytopenic purpura (ITP) refractory to steroids and immunoglobulins who achieved complete normalisation of their platelet counts after treatment with rituximab, 375 mg/m2 given weekly in four doses...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.