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Haemoglobin Bristol-Alesha in a child with non-spherocytic severe haemolytic anaemia and marked anisochromic poikilocytosis with basophilic stippling and amorphous intracellular content.
Blood Cells Mol Dis
May 2022
Centre for Haematology, St Mary's Hospital, Campus of Imperial College, London, United Kingdom of Great Britain and Northern Ireland.
Here we describe a retrospective study of a 10-year-old girl, adopted from India, and referred to the Rare Anemias Unit for the diagnosis of a severe haemolytic anaemia of unknown etiology. Blood film examination revealed markedly abnormal red cell morphology characterised by a mixture of very pale (hypochromic) cells with basophilic stippling and macrocytic cells containing coarse basophilic dots and an amorphous material of unknown origin. With a presumptive diagnosis of pyruvate kinase deficiency (PK), the patient had been splenectomised at 7 years of age with a partial recovery of the anaemia and a decrease of the blood transfusion rate.
View Article and Find Full Text PDFSplenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment.
Am J Hematol
January 2022
Service de Médecine Interne et Immunopathologie-IUCT Oncopole, Centre Hospitalier Universitaire de Toulouse, Toulouse, France.
Although splenectomy is still considered the most effective curative treatment for immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the approval of thrombopoietin receptor agonists (TPO-RAs). The main objective of the study was to determine whether splenectomy was still as effective nowadays, particularly for patients with failure to respond to TPO-RAs. Our secondary objective was to assess, among patients who relapsed after splenectomy, the pattern of response to treatments used before splenectomy.
View Article and Find Full Text PDFPractical Recommendations for the Management of Patients with ITP During the COVID-19 Pandemic.
Mediterr J Hematol Infect Dis
May 2021
Istituto di Ematologia "Seràgnoli", IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
The current COVID-19 pandemic requires revisiting our current approach to major blood disorders, including ITP (Immune Thrombocytopenia), stirring up the production of several disease-specific practical guidelines. This report describes an updated version of consensus-based practical guidelines on the management of ITP, adapted to the Italian health system and social context. It highlights the role of the hematologist in offering guidance for choosing differentiated approaches in relation to specific circumstances and is intended to provide them with a useful tool for sharing the decision-making process with their patients.
View Article and Find Full Text PDFDelayed Splenic Rupture: A Rare Complication of Snakebites.
Wilderness Environ Med
March 2021
Department of Emergency Medicine, Yonsei University Wonju College of Medicine, Gangwondo, Republic of Korea. Electronic address:
Bites by venomous snakes can cause fatal systemic and coagulation disorders. Rare complications, such as compartment syndrome and hemoperitoneum, may also require surgical intervention. Here, we describe our experience with an unusual case of snakebite-induced delayed splenic rupture.
View Article and Find Full Text PDFInitial modelling and updates on cost effectiveness from the first 10 years of a spleen registry.
Aust N Z J Public Health
October 2018
Deakin Health Economics, Deakin University, Victoria.
Objective: To validate our estimates from our original model and re-evaluate the cost-effectiveness of Spleen Australia, the Australian post-splenectomy registry, using our original model with updated model parameters based on advances in the literature and experience of the registry over the past decade.
Methods: We revisited a decision model from 2005, comparing 1,000 hypothetical registered patients with asplenia or hyposplenism against 1,000 who were not registered, and updated the model parameters. The cost-effectiveness of the registry was evaluated from a healthcare perspective in terms of additional cost per case of overwhelming post-splenectomy infection (OPSI) avoided and as additional cost per life year gained.
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