Developmental aspects of splenic function in sickle cell diseases

Abstract

In order to study developmental aspects of splenic function and dysfunction in children with Hb SS disease and other Hb S syndromes. red cells were examined by interference phase-contrast microscopy. In

Improved Splenic Function After Hematopoietic Stem Cell Transplant for Sickle Cell Disease

“…The two techniques used to evaluate splenic function in this study, 99m Tc sulfur colloid LS scans and RBC pit counts, have been previously well studied in SCD. [1][2][3]22,[46][47][48] Similar to the BABY HUG trial, we found that decreased uptake on LS scan does correlate with an increased RBC pit count. [3] An important strength of our study is that all LS scans were centrally reviewed by a single individual masked to pre-/posttransplant status to prevent inconsistencies and bias in scan interpretation.…”

“…Splenic injury begins in infancy and most individuals with hemoglobin (Hb) SS have functional asplenia by 3 years of age. [1][2][3] Without a functioning spleen to help remove microorganisms, bacteremia can progress rapidly. Historically, infection from encapsulated bacteria was the major cause of death for children with SCD.…”

Incidence of invasive Haemophilus influenzae infections in children with sickle cell disease

“…Children with sickle cell disease (SCD) are at increased risk for invasive encapsulated bacterial infections such as Haemophilus influenzae due to impaired splenic function. 1,2 The advent of prophylactic peni- 4 The appropriate acute management of such infections as well as long-term infection prophylaxis strategies for children with SCD after H. influenzae infection is not well defined. 5 Prior to the introduction of penicillin prophylaxis and vaccination against Hib, the incidence of systemic infection prodrome in most children with H. influenzae bacteremia, mortality rate for invasive infection was high at 20% to 30%.…”

Prophylactic penicillin after 5 years of age in patients with sickle cell disease: A survey of sickle cell disease experts

“…The risk is greatest in individuals with homozygous sickle cell anemia (HbSS) and sickle b 0 thalassemia (HbSb 0 ) but may also be increased in sickle hemoglobin-C disease (HbSC) [3,4]. Functional asplenia, which occurs commonly by 2 years of age in children with HbSS and HbSb 0 but infrequently in children under 5 years of age with HbSC, causes the risk for invasive pneumococcal disease (IPD) in SCD [5,6]. The Prophylactic Penicillin Study (PROPS) established prophylactic penicillin as standard care for children with HbSS and HbSb 0 under 5 years of age [7].…”