functional hyposplenia

Last reviewed 01/2018

  • splenic hypoplasia is a poorly defined and infrequently recognized condition that is usually not associated with other anomalies and may be familial
  • functional asplenia is associated with conditions such as homozygous sickle cell disease, hemoglobin sickle cell disease, and sickle cell hemoglobin (Hb S) beta thalassemia
    • most children with these hemoglobinopathies are functionally hyposplenic starting in the first year of life and become anatomically asplenic (due to splenic infarction and splenic atrophy) by the second decade of life
    • infection risks in these individuals parallel those of patients with asplenia
    • patients who undergo splenectomy because of thalassemia or Hodgkin disease have a higher risk of overwhelming infection than those patients with functional hyposplenia secondary to sickle cell disease
    • additional conditions associated with splenic hypofunction include neonatal age, rheumatologic diseases (systemic lupus erythematous (SLE), rheumatoid arthritis), inflammatory bowel disease, graft versus host disease, coeliac disease and nephrotic syndrome