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Parvovirus associated aplastic crisis in homozygous sickle cell disease
Insights
Human parvovirus outbreaks cause aplastic crises in children with sickle cell disease in Jamaica. Prompt diagnosis and management ensure a good prognosis for this self-limiting condition.
Area of Science:
- Hematology
- Pediatrics
- Virology
Background:
- Aplastic crises are a significant complication in children with homozygous sickle cell disease.
- These crises can occur epidemically, particularly in specific geographic regions.
- Human parvovirus is a known trigger for aplastic crises.
Purpose of the Study:
- To investigate the incidence and characteristics of aplastic crises in Jamaican children with homozygous sickle cell disease.
- To determine the role of human parvovirus in these aplastic crises.
- To describe the clinical presentation, management, and outcomes of affected children.
Main Methods:
- A cohort study was conducted on 314 children with homozygous sickle cell disease in Jamaica.
- Data were collected on cases of aplastic crisis, including age, etiology, symptoms, and management.
- Statistical analysis was used to determine incidence and associations.
Main Results:
- Of 314 children, 67 experienced aplastic crises, with 62 linked to human parvovirus infection.
- The incidence of parvovirus-associated aplastic crisis reached 28% by age 10.
- Symptoms were primarily due to viremia and acute anemia; asymptomatic thrombocytopenia was common. Blood transfusion was administered in 87% of cases.
Conclusions:
- Human parvovirus is the predominant cause of aplastic crises in Jamaican children with homozygous sickle cell disease.
- Aplastic crisis associated with parvovirus is a self-limited condition.
- Prompt diagnosis and appropriate management lead to an excellent prognosis.
Abstract:
Aplastic crises in homozygous sickle cell disease in Jamaica predominantly affect children and occur in epidemics. Of 67 cases in a cohort study of 314 children with homozygous sickle cell disease, 62 were attributable to human parvovirus infection. Affected children were aged 0.5-12.5 years, and the incidence rose to 28% by 10 years. No recurrences were seen. Symptoms and signs on presentation were attributable to the viraemia and acute anaemia. Asymptomatic thrombocytopenia was common. Blood transfusion was given in 54 cases (87%). Thirty eight children (61%) were admitted to hospital, 16 of whom were extremely ill on presentation and one of whom died soon after admission. Twenty four (39%) were managed as outpatients, 16 of whom were transfused. Parvovirus associated aplastic crisis is a self limited condition with excellent prognosis if diagnosed promptly and managed appropriately.