Emergency red cell exchange for the management of acute complications in sickle cell disease: Automated versus manual.

Background: Red blood cell exchange is the cornerstone of the management for acute complications of sickle cell disease. It improves anaemia and improvesperipheral tissue oxygen delivery while at the same time reduces the proportion of circulating sickle erythrocytes. Even though automated red cell exchange is very effective in rapidly lowering the Hb S level, 24-h availability is currently not feasible for most specialist centres including our own.

Mortality Rates and autopsy findings in fat embolism syndrome complicating sickle cell disease.

Fat embolism syndrome is a rare but underdiagnosed complication of sickle cell disease associated with high morbidity and mortality. It affects predominantly patients with a previously mild course of their illness and those of non-SS genotypes while there is possibly an association with infection with human parvovirus B19 (HPV B19). Here, we present the mortality rates and autopsy findings of all reported cases to date.

Pitfalls in Diagnosing Thrombotic Thrombocytopenic Purpura in Sickle Cell Disease.

Thrombotic thrombocytopenia purpura is characterised by microangiopathic haemolytic anaemia and red cell fragmentation on the peripheral smear, neurological involvement and thrombocytopenia. Diagnosis in the context of sickle cell disease can be challenging due to the inherent haemolytic state and the multitude of other associated complications of the latter. Specifically, fat embolism syndrome characterised by respiratory failure, neurological impairment and thrombocytopenia can be misdiagnosed this way.

Rate of Dental Extractions in Patients with Sickle Cell Disease.

Background: Sickle cell disease is an inherited disorder associated with chronic haemolysis and anaemia, recurrent episodes of pain and potentially multisystem end-organ damage. A lot less is known about the dental health of these patients.

Aims: To explore the incidence of severe dental disease leading to dental extraction in our sickle cell population.

Therapeutic plasma exchange in the management of acute complications of sickle cell disease: A single centre experience.

Sickle cell disease results in systemic inflammation even at steady state and this is accentuated during acute crises. The plasma of affected patients contains several proinflammatory cytokines as well as adhesion molecules and prothrombotic factors. This environment promotes further red cell sickling while many of these substances can cause direct tissue toxicity and end-organ damage.

Complete neurological recovery from fat embolism syndrome in sickle cell disease after sequential red cell exchange transfusion and therapeutic plasma exchange.

Fat embolism syndrome in sickle cell disease is associated with great mortality, while more than half of survivors suffer severe neurological sequelae. Release of fat droplets leads to obstruction of the microcirculation as well as generation of proinflammatory cytokines that can cause direct tissue injury. Red cell exchange transfusion can be life-saving but the addition of therapeutic plasma exchange may further improve outcomes by removing such inflammatory mediators.