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, haemoglobin sickle cell disease, and sickle cell haemoglobin (HbS) 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
Reference
Kirkineska L et al. Functional hyposplenism. Hippokratia v.18(1); Jan-Mar 2014.
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