Severe refractory idiopathic warm autoimmune haemolytic anaemia responsive to complement inhibition with eculizumab.

We report a case of severe idiopathic warm autoimmune haemolytic anaemia (wAIHA) which was initially poorly responsive to treatment with corticosteroids, intravenous immunoglobulin, mycophenolate mofetil and rituximab, and required transfusion of more than 30 units of red cells over 12 weeks. Off-label use of the terminal complement pathway inhibitor, eculizumab, led to rapid amelioration of the haemolysis, presumably by the inhibition of an intravascular component, and allowed time for slower acting immunosuppressive agent to take effect. This novel approach warrants further evaluation, given the poor prognosis of multirefractory wAIHA.

Salmonella pyomyositis complicating sickle cell anemia: a case report.

INTRODUCTION: Pyomyositis is a bacterial infection of skeletal muscle and a rare complication of sickle cell anemia. It may present a difficult problem in diagnosis, leading to delay in appropriate treatment and development of complications including abscess formation and osteomyelitis. CASE PRESENTATION: We report the case of a 44-year-old Afro-Caribbean woman with homozygous sickle cell disease who presented with chest crisis and later developed pyomyositis of her hip and pelvic muscles. Salmonella agbeni was isolated from blood cultures and magnetic resonance imaging confirmed the diagnosis in this case. It is noteworthy of this case that there were no antecedent signs of gastroenteritis. Drainage was not appropriate and she was treated with intravenous antibiotics for six weeks. CONCLUSIONS: Focal Salmonella infections are uncommon in soft tissue. Pyomyositis should be considered in patients with sickle cell anemia that continue to have muscle pain and high fevers, despite initial management of their sickle cell crisis. Radiological imaging, particularly magnetic resonance imaging, is a crucial tool in establishing the diagnosis.