Kostmann's syndrome is a rare congenital disorder of neutrophil production due to impairment of myeloid differentiation in the bone marrow, with the neutrophil count being characteristically less than 500 x 10(3) cells/l (normal: 2-7 x 10(9)/l). Severe persistent neutropenia results in an increased susceptibility to frequent bacterial infections. The condition can be treated with recombinant human granulocyte colony-stimulating factor (G-CSF). Although several articles have addressed the clinicopathological and haematological aspects of this disorder, little or no information has been available concerning the radiological findings in this disorder. This report summarizes the clinical features, radiological findings and management of a patient with Kostmann's syndrome.
INTRODUCTION: There is an awareness of the increased incidence of splenic abscess in Southeast Asia giving rise to unexplained fever. This study looks at the role of computed tomography (CT) in evaluating focal splenic lesions in patients presenting with fever.
METHODS: 37 patients presenting with fever of unknown origin underwent CT and this study retrospectively analyses the findings in these patients. 13 patients also had associated abdominal pain. Patients with conditions at high risk for splenic infection include: diabetes mellitus in ten patients, leukaemia in seven patients, human immunodeficiency virus infection in five patients, intravenous drug abuse in six patients, and steroid therapy in two patients. No risk factors could be identified in seven patients.
RESULTS: Splenic abscess was diagnosed in 28 patients. A range of infecting organisms was isolated but the most frequent were Staphylococcus aureus (eight), tuberculosis (four), Streptococcus (four), fungal (four) and melioidosis (four). No infecting organism could be identified in ten cases though in patients with leukaemia with multiple low attenuation areas, the cause was presumed to be fungal. Six patients were diagnosed to have splenic infarcts though differentiation from splenic abscess could be difficult; these patients were treated for an abscess and all had endocarditis. Three patients were subsequently diagnosed with lymphoma. Percutaneous abscess drainage was performed in five patients and splenectomy was carried out in six patients.
CONCLUSION: CT proved to be very useful as it not only revealed the size and extent of any splenic abnormality but it assisted with guidance for percutaneous drainage, determined the site for biopsy, and provided follow-up after treatment.